k Croup U22 Neurology Prepared by Group 433 (2007) GENERAL PART 1) Peripheral nervous system. Roots of the spinal cord (anterior and posterior), Plexuses (cervical, brachial, lumbar). Disorder of nerve roots and peripheral nerves, Function of PNS ~ Carry impulse to and from the CNS. It regulates motor, sensory and autonomic action It consists of Cranial and Spinal nerves (from the point of exit from CNS to their termination in peripheral structure). Anatomy : . _ ——_—____.Spinal PNS Cra ~ Ant and post horns ~~ Granial nerves Ventral and dorsal roots ~—Its muclei & roots +Dorsal root ganglia Spinal nerves Plexus. “Peripheral nerves ~ Each segment of SC gives ventral (motor) arid dorsal (sensory) roots on each side. ~ These unite in the intervertebral foramina to leave the spinal canal, a mixed spinal nerve (31 pairs). ~The spinal nerves proper are very short and split into dorsal and ventral rami, Dorsal ram serve the post body trunk; ventral rami (except TI-T12) form plexus (cervical, brachial lumbar, and sacral) that serve the limbs. ~ Brachial plexus (C5-T1) supply the upper limbs and lumbosacral plexus supply the lower limbs. ~ Peripheral nerve (PN) is composed of motor, sensory and autonomic axons. ~ PN dysfunction may result from damage to the nerve fibers, cell body, myelin sheath, DNS Lesion Neuropay A syndrome of sensory loss, muscle weakness and atrophy, [deep tendon reflexes, vasomotor symptoms, alone or in combination Classification 1) Mononeuropathy: Single peripheral or cranial nerve lesion 2) Radiculopathy : Lesion of roots = — 3) Plexopathy : Plexus lesion -9 ie teexr Plees + 4) Polyneuropathy: Symmetrical PN lesions that may affect many nerves simultaneously Neuropathy sympto 1) Peripheral paralysis signs: Weakness of muscles, atonia, atrophia, loss of reflex(areflexia) 2) Sensory symptoms: Pain, loss of sensation, complain pins and needles sensation 3) Vasomotor symptoms: Cold blue extremities, skin dryness, hyperkeratosisNeurology Prepared by Group 433 (200 Plexus Syndrome = Brachial plexus lies on the post triangle of the neck Between the m. seal medius. ~ It includes C5-T1 roots. Nerves leaving from the brachial plexus supply the upper limbs. - Brachial plexus syndrome is caused by: ¢ Trauma (fracture, dislocation of shoulder and clavicle) * Fraction on the arm at birth (infants) ¢ Infiltration by malignant tumours * ‘Diabetes ant and scale: Upper plexus.lesion (C5-C6): Erb-Duchenne Paralysis - When upper parts of the brachial plexus are affected, weakness and atrophy of shou! girdle and proximal arm muscles occurs. Biceps jerk is absent. Lower plexus lesion (C8-T]): Klumpke's Paralysis - Injuries to the lower brachial plexus producing distal arm weakness and wasting and fo sensory deficit in forearm and hand. - Horner's syndrome may be a feature Total brachial lesion - This results in complete flaccid paralysis and anesthesia of the acm. ~ Presence of Homer’s syndrome — proximal T{ nerve root involvement. Upper limb mononeuropathies Etiology - Fractures of humerus, forearm - Prolonged pressure (Saturday night palsy) - Compression in the forearm & wrist (Carpal tunnel syndrome) ~ Neoplastic infiltration - Lipoma, fibroma, neuroma - Systemic causes (diabetes) - IMinjections Radial Nerve Symptoms = Weakness of the wrist and finger extensor (wrist drop with flexed finger) = Sensory loss on dorsal part of hand & forearm - Loss of triceps and supinator reflexesNeurology Prepared by Group Median Nerve Symptoms (C7C8) Due to injury in axilla - compression in forearm, at the wrist (Carpal tunnel syndrome) Weakness of abduction and apposition of thumb Weakness of pronation of the forearm Deviation of wrist to ulnar side on wrist flexion Weakness of flexion of distal phalanx of thumb and index finger Wasting of thenar muscles Severe burning pain in the wrist and palm Sensory loss on index and middle finger Ulnar Nerve Symptoms (C7-C8, = Weakness and wasting muscle supply (adductor pollicis, dorsal interosseous, thumb abductor) - Characteristic posture of hand -‘claw hand’ deformity - Sensory loss on the 5" finger, ulnar aspect 4" fingers and ulnar border of the palm. ‘umbosacral Plexus Syndrome -tiology Fractures of the pelvic bones Neoplastic infiltration Compression from an abdominal mass = Psoas muscle abscess - Systemic causes (diabetes) Lumbar Plexus > - Includes anterior roots of T12-L5 segment, the plexus located in the psoas muscle + Important branch are femoral and obturator nerves - Lumbar plexus lesions produce: * Weakness of hip flexion © Weakness of knee extension with wasting of thigh muscles © Knee jerk is lost . Sensory loss over the anterior and middle aspects of thigh Includes a }-S3 roots, the plexus is located in the posterior wall ofthe ars ~The L4-Sd divisions form the common peroneal ngzve. ( {isct 45 - The L4-S3 anterior division forms the tibial nerve. ae P —+ Both these nerves fuse to form sciatic nerve, - Sacral plexus lesions produce: © Weakness of posterior thigh (hamstring) and foot muscle * Posterior leg sensory lossNeurology Prepared by Group 43 * Congenital or traumatic hip dislocation * Penetrating injuries * Entrapment of sciatic notch Symptoms: © Weakness of hamstring muscles with loss of knee flexion e Ankle-reflex is absent © Sensory loss of outer aspect of leg Common Peroneal Nerve (L4-L35, ‘The nerve arises from the division of the sciatic nerve in popliteal fossa. Damaged by: + Trauma to the head of the fibula, pessing here from kneeling, leg crossing * Systemic causes (diabetes) Symptoms: ‘+ Weakness of dorsiflexion and eversion of the foot © Patient can’t stand on heel © Walking with “foot drop” ‘* Sensory loss of the dorsum and outer aspect of the foot Posterior Tibial Nerve Etiology: Trauma in popliteal fossa « Fracture of tibia * Systemic cause (diabetes) Symptoms: * Weakness of plantar flexion and inversion of the foot Patient cannot stand on toes Ankle reflex is lost Sensory loss on sole of the foot Pain in ankle and sole of foot .Neurology Prepared by Group 433 (2 2) Grey matter of spinal cord. Symptoms of lesion (anterior and posterior horns) on different level. . Grey matter of sp’ cord (SC) = Grey matter of SC consists of nerve cells grouped to form nuclei, > General pattern of grey matter forms the letter “H1” against the background of the white matter, + Grey matter around the ceatral canal is termed the substantia intermedia centralis, ~ Ineach column of grey matter, 2 columns are distinguished: _* Columna anterior © Columna posterior ~ On transverse section, they are seen asa dilated anterior horn and a pointed posterior horn, {Posterior | - Contain somatic and autonomic sensory nuclei. horns * Nucleus thoracicus (Stilling-Clarke column) * Substantia gelatinosa | |___* Nuclei proprii ee _ Anterior |= Contain cell bodies of somatic motor neurons and mofor nuclel, which provide nerve horns impulses for contraction of skeletal muscle. * Their short columns located ia 2 groups: medial and lateral, * Medial group innervate muscles developing from the dorsal part of the myotomes. + Lateral group innervate muscles derived from the ventral part of the myotomes. ‘eral ~ Present only in the thoracic, upper lumbar and sacral segments of the spinal cord. ns ~ Contain cell bodies of autonomic motor neurons that regulate activity of smooth muscle, cardiac muscle and glands, Symptoms of anterior horns lesion on different level When the anterior horns lesion, the lower motor neuron (LMN) weakness appear: 3A+1F symptoms: Atonia - Hypotonia with diminished resistance to passive stretch | - Muscle tone is tested (controlled) through the passive movement of | _extremities, ; | Atrophia “| Wasting becomes evident in the paretic muscle within 23 weeks of ihe onset r (muscle wasting) ot _ Areflexia Depressed or absent deep reflexes tendon and periostal. | Musete fasciculation | Involuntary contraction or twitching of muscle fibers v iin, Pathology Plegia = Nbsent oF active movement and power * Monoplegia — Only one limb is involved, ‘* Hemiplegia ~ Both right limbs or both left limbs are involved. * Paraplegia ~ Both upper or both lower limbs are involved. * Paresis. ~ Not complete but partial |of muscle power and active movement, wNeurology Prepared by Group 433 (2007 @) t,mamillar tine _A\ ‘t; thoracic arch @ | Tounbilicus Tu. Ly inguinal fold Lr Lumbar segments & roots Si-Ss-Cor2 | | Sacral and coceygeal & roots segments Cervical segments & roots Thoracic segments & roots foe en po LyLs 4 . ff . Clinical features C1-C4 ~ Central tetrapalysis ~ Anaesthesia below the lesion level =_ Sphincter disorder (faecal and utine retention) 5-72 = Peripheral upper limbs palsy ~~ Central lower limbs palsy - Anaesthesia below the lesion level =_ Sphincter disorder (faecal and urine retention) T3—T12 | Central lower limbs palsy - Anaesthesia below the lesion level -_ Sphincter disorder (faecal and urine retention) T1-S2 > Peripheral lower limbs palsy - Anaesthesia below the lesion level = Sphincter disorder (faecal and urine retention) S3-85 = Palsy is absent - Sensory impairment in “saddle” area Incontinence of urine and faccals (detrusor paralysis) - Alesion: above ‘the Li vertebral body may damage both cord and root, below LI, only are damaged. CG Sphincter disorder due to S3-S5 innervations is affected. * IFLMN weakness, sphincter become atonia so patient has incontinence. ‘If UMN weakness, sphincter become spastic/hypertonia so patient has retention of w feces.‘Neurology Prepared by Group 433 (2007 ¢-S¥mptoms of posterior horns lesion on different level Posterior horns lesion — Sensory impairment Negative symptoms: a [ Hypoaesthesia Decreasing (loss) of | Anaesthesia Lack of feeling | Anaelgesia Loss of pain sensi 'Thermanaelgesia_| Loss of temperature sensation ysnesthesia- ‘Wrong sensation uch of hot objects is pere “casory ataxia | Impairment of deep sensation (proprioreception) due to impaired joints position sensation: - Open eyes : The gait appears more normal - Closed eyes: The gait will be worse ~The patient falls to one or both sides and swing in Romberg’ position - The gait is broad based and reeling, like “sailor's gait” or “drunkard’s gait” Positive symptoms: Hyperaestesia_| t of feeling —— Feeling of pins and needles sensation or burning feeling without irritation (touching object) Result of sensory fibers irritation = Intense, continuous, burning pain produced by an incomplete peripheral nerve injury. - Touching the limb aggravates the pain, and the patient resents. any interference or ; attempt at limb mobilization, The skin becomes red, warm and swollen. ! - Only occurs with damage to peripheral nerves containing a large number of sympathetic fibers and responds in part to sympathetic blockade (pharmacological or surgical), ‘Types of sensory impairment 1) Peripheral type Impairment of all kinds of sensation in the zone of impaired nerve, plexus, and spinal root innervation, The pattern of the sensory deficit aids lesion localization (nerve, root, and plexus) Eg. Mononeuropathy (n. ulnaris), Spinal root (L5), Plexus brachialis 2) Polyneuropathic type ~ Sensory loss of a “socks and gloves” (both superficial and deep) due to multiply damage of peripheral nerves of distal parts of limbs. - It may accompany exogenic and endogenic polyneuropathies. ¢ Exogenic — Alcoholism, intoxications = Endogenic — Diabetes, nutritional, hepatotoxic and so on.Neurology Prepared by Group 433 (20¢ 3) Conductive type Impairment of all sensory modalities below the level of damage (in brain stem; spinal cord). 4) Syringomyelitic type (dissociated = Due to the damage of spinal posterior horns. Loss of pain and temperature sensation with retention of other senses, occurring in a “cape-like” distribution - Painless burns are a classic sign. Syringomyelia is a result of pathological development spinal cord (antenatal). ~ The cavitation appears inside the spinal cord, normally in the place of posterior horns. 5) Brown-Sequard syndrome (stage of extramedullar tumour) - Loss of all modalities at the one or several dermatome level ipsilaterally. - Loss of pain and temperature (superficial) sensation below a specific dermatome lev contralaterally. - Loss of proprioreception (deep sensation) up to similar level and limb weakness ipsifaterallyNeurology Prepared by Group 433 (2007) 3) siti cnsaes of lesion on different levels. Gray matter ‘White matter 1. Antarior horn 4, Anterior funiculus 10. Contrat canat 2. Posterior horn 5. Lateral funieulus 11, Antorior root 3. Gray commisure 6. Posterior funsculus 12, Posterior root 7. Anterior commisura 48. Dorsal root ganglion 8. Antorior median fissure 8. Posterior modian sulcus Structure ~ Extends from the MO in the brain and continues to the conus medullaris near the lumbar level at L1-2, terminating in a fibrous extension known as the filum ferminalé~ *) - ~45 om Tong in men and 43 cm long in women, ovoid-shaped, and is enlarged in the cervical and lumbar regions, ~ The peripheral regions —> white matter tracts containing sensory and motor neurons, + The central region is four-leaf clover shaped/butterfly shaped (gray matter) that surrounds the central canal (an anatomic extension of the 4" ventricle) and contains nerve cell bodies. - The 3 meninges that cover the spinal cord: * the outer dura mater, * the arachnoid membrane, and « the innermost pia mater - The meninges are continuous with that in the brainstem and cerebral hemispheres, with CSF found in the subarachnoid space. a ~ The cord within the pia mater is stabilized within the dura mater by the connecting denticulate ligaments which extend from the pia mater laterally between the dorsal and ventral roots. °Neurology Prepared by Group 43: Spinal cord segments - 31 different segments, with motor nerve roots exiting in the ventral aspects and sens nerve roots entering in the dorsal aspects, ~The ventral and dorsal roots later join to form paired spinal nerves, one on each side of spinal cord + There are 31 spinal cord nerve segments in a human spinal cord: * 8 cervical segments © 12 thoracic segments * 5 lumbar segments 5 sacral segments 1 coceygeal segment ~ Because the vertebral column grows longer than the spinal cord, spinal cord segme become higher than the corresponding vertebra, especially in the lower spinal cord segme- in adults, - Ina fetus, the vertebral levels originally correspond with the spinal cord segments ~ In the adult, the cord ends around the L1/L2 vertebral level at the conus medull of the spinal cord segments located superiorly to this. ‘There are two regions where the spinal cord enlarges: a ae Cervical * Corresponds roughly to the brachial plexus nerves, which innervate the enlargement limb. Includes spinal cord segments from about C4 to TI, and is fo vertebral levels of C4 to TI. _ Lumbosacral | ¢ Corresponds to the lumbosacral plexus: nerves, which innervate tic enlargement limb. * Comprises the spinal cord segments from 2 to S3, and is found about vertebral levels of T9 to T12 ° ys | Anaesthesia below the lesion level I Sphincter disorder (faecal and urine retention) Peripheral upper limbs palsy - Central lower limbs palsy Anaesthesia below the lesion level 7 Sphincter disorder (faecal and urine retention) T3-T12 |= Central lower limbs palsy Anaesthesia below the lesion level Sphincter disorder (faecal and urine retention) L1—S2_ | Peripheral lower limbs palsy Anaesthesia below the lesion level Sphincter disorder (faecal and urine retention) $3 =85 ~ Palsy is absent - Sensory impairment in “saddle” area Incontinence of urine and faecals (detrusor paralysis) _ C5 —T2Neurology Prepared by Group 433 (2007) iP 4) Medulla oblongata. Symptoms of lesion. Bulbar and pseadobulbar palsy. ~ Medulla oblongata (MO) or myelencephalon is a direct continuation of spinal cord into brain stem. —T ~ Shape like bulb, ~ Supetiorly Sittated pons varolli, inferiorly situated roots of C1 and foramen magnum - Consist of structures: * Nuclei of CN 1X, X, XI, XL + Olivary nucleus (balance) nucleus CCN, loytt/12) © Reticular formation VesPrredory andl civeulateryPeerk. co Respiratory and circulatory centers Function; Regulation of respiration, heart rate and blood pressure ——— ts ——x mptoms of lesion ; Motor I Sensory | Contralateral - UMN weakness ~~~ unilateral |- Facial sensory lose on opposite site lesion hemiplegia. - Loss of pain and temperature on ~ Pain and temperature loss on same opposite side of face with or without side as the weakness, ‘muzzle’ area sparing. Homer’s syndrome (ptosis, miosis, | - Hemisensory loss of body. sweating disturbance) on opposite |- Loss all modalities in limbs side, depending on extent of lesion. - Weak palate and tongue. on }- Weakness of palate and tongue on ‘opposite side. side opposite to limb sensory deficit Bilateral lesion | - UMN weakness ~ tetraplegia» : Facial. movements retained, but no | tongue or palate movement or speech, Etiology of lesion wu yi + Infarction - Demyelination | - Syringobulbia ~ Poliomyelitis - Tumor - Motor rieuron diseases nucleus CW C9,l0N)12) RePirafoly and erreuatsy centers Symppen of Fran f weapnes lum) , loss pain anol Few fret Saf’ al shorners Synd Ome. Cpe anjos/s » SMbating Ai stulovh|’ depletion, Ach excess): Result: Hypertonic | CHOREA hypokinetic syndrome > PARKINSONISM. Hivpotonic-Hvperkinetic Syndrome 7" 11 2 Fe a merhlnee See ZS / * Involuntary, non-repetitive, imegular jerking movements affecting limbs and axial muscle groups. * Swift grimaces and sudden movements of head or limbs, * Causes: Neurorheumatism (Sydenhai jorea}, Huntington’s disease, antiparkinsonic drugs, encephalitis, hyperthyroidism, hypokalemia} alcohol. 2) Athetosis © Inegular, slowing, sinuous writing movement involving the distal parts of the limbs, * Results from: Hypoxic neonatal brain damage, lipid storage disorder. 3) Dystonia ce ‘Slow, sustain sustainec abnormal movement. movement. Ais unelnet norma] move.) © Generalized dystonia with abnormal posturing of the head, trunk and limbs (idiopathic torsion dystonia). © Partial (Focal): Eg. Unilateral deviation of the head caused by dystonic contraction of the m sternocleidomastoidus. (Localized) cramp- Due to hard walk, cold, dystonia of carpopedialis, writing spasms.Neurology Prepared by Group 433 (21 4) Ballismus * Explosive, violent movement. * Hemisbalismus: Involves one side of the body, usually the proximal extremity mus (the limb suddenly flies about in all directions out of control). ‘Abrupt, jerky movement affecting the head, neck and trunk. * Can be voluntarily suppressed and often take form of winking, grimacing, shou! shrugging, sniffing and throat clearing. 6) Myoclonia © Shock-like contraction of muscles which occur irregularly and asymmetric repetitively to the same group of muscles (flinch, wince). pokinetic Syndrome (Parki 1) Rigidity Extrapyramidal muscle hypertonicity © Stiffness felt by examiner when passively moving limb. © There are 2 types of rigidity: © Plastic or “lead pipe” (steady increase in resistance) © “Cogwheel” (ratchet-like increase in resistance) 2) Hypokinesia — Loss of voluntary movements ¢ Bradykinesia: Slowness in voluntary movements * Oligokinesia: Movement number | © Acheirokinesia: | arm swinging amplitude when walking © Shuffling gait: Small rapid steps to keep up with centre of gravity e Flexed, bent posture Hypomimia (masked-like face), often with drooling Reduced blinking 3) Tremor j © At rate of 4-8/sec, “pill-rolling” coarse in character, distally, thumb moving rhythmical Occurs at rest, improves with movement and disappears during sleep. © Begins unilaterally in upper limbs and eventually spreads in all 4 limbs. 4) Postural Disturbance * Flexion of limbs and trunks is associated with a failure to make quick postural or wri adjustments to correct imbalance. © Patient falls when turning or if pushed (propulsion, lateropulsion, retropulsion).ULAE, iat Neurology postal Cor? Prepared by Group 433 (2007) 10) Hemisphere of brain. Anatomy, white and grey matter. Lobes and borders. Dominant and non-dominant hemisphere. Centre of speech, sensory, motor and other system, - The brain can be divided into left and right cerebral hemispheres, ~ Right and left cerebral hemispheres are separated be a deep Qidtine sagittal fissure)— the longitudinal cerebral fissure. + In depth of the fissure, the corpus callosum connects the hemisphere across the midline 4 of these hemispheres has an outer layer of grey matter called the cerebral cortex that is wted by an inner layer of white matter. < Inatter of hemisphere consists of a great number of nerve fibres stretching in different sections and forming the conduction pathways: 2 [Associated fibers | Conduct nerve impulse between gyri in the same hemisphere, - Commissural fibers Conduct nerve impulses from gyri in one cerebral hemisphere to corresponding | gyti in the other cerebral hemisphere v | ‘Projection fibers ‘Conduct nerve impulses from the im to fower parts of the CNS or from _ the lower parts of the CNS to cerebrum. ~ The cerebral cortex has su/ci (small grooves), fissures (larger grooves) and bulges between the » grooves called gyri. - 3 surfaces of cortex are distinguished: 1. Superiolateral surface of hemisphere (convex) 2, Inferior surface of hemisphere (basic) 3. Medial / internal surface of hemisphere Superiolateral surface of hemisphere: + Each hemisphere has 4 main lobes: frontal, temporal, parietal and oceipital lobe. + In order to increase surface-area of cerebral cortex: maximally, surface of each cerebral hemisphere is thrown into folds or gyri, which are separated from each other by sulci or fissure. - Sulcus Centralis, (Rptando's) ‘Sulcus Parietooccipitalis a 4 f Sy (Fron Lobus \ lobe Parietal i Occipital fobs \ Soleus Lateral pr att Fa ) ous Laeralis SW) ( f \ Lobus Temporalis, 4 ea 21Neurology Prepared by Group 433 (2 Medial / internal surface of hemisphere Corpus cailosum, cerebrum, lateral ventricle, 3 ventricle, diencephalons (hypothalamus and thalamus), pituitary gland, 4" ventricle, brain stem (midbrain, pons, medulla oblongata), cerebellum, reticular formation. inferior surface of hemisphere (basic) Bulbus. olfactory, olfactory: tract, chiasma opticus, brain (midbrain, pons, medulla oblongata), middle cerebellar ped cerebellum. - Every specific part of cerebral hemisphere is responsible for a central aspect of function. - Innormal circumstances, these functions are integrated and the patient operates as a whole. ~ Damage to part of cortex will result in characteristic disturbance of function. - Brodmann and Betz described the basic function of cortex. Dominant and non-dominant hemisphere ~ Unilateral brain damage reveals a difference in function between hemispheres. - Left hemisphere is dominant in all right handed people. - Left handed people subjects the left hemisphere is dominant in the majority (up to 75%). Dominant hemisphere + Language (speech) * Math + Face recognition + Logic + Visual imagery (non-language depend + Language dependent memory memory) + Reading, writing, gnosis, praxis + Music> Neurology Prepared by Group 433 (2007) ~ Hemisphere dominance may be demonstrated by the injection of sodium amytal into the internal carotid artery. ~ On the dominant side, this will produce an arrest of speech for up to 30 seconds — WADA test ~Such a test may be important before temporal lobectomy for epilepsy when handedness/hemisphere dominance is in doubt. kore lip Lag rd iow forge Z swacrisarum Imac eta = ~Saleaton 04 ae entre of speech, sensory, motor and other system Centre of speech * Broca's area is in the lower part of the frontal lobe and is involved in the formation of sentences * Wernicke's area, located in the temporal lobe is.involved in the comprehension of speech, Centre o1 ry Sensory impulses arrive mainly in the posterior half of both cerebral hemispheres, in regions behind the central sulci, Primary - Located directly posterior to the central sulcus of each cerebral somatosensory area | hemisphere in the postcentral gyrus of each parietal lobe. (areas 1,2,3) ~ It extends from the lateral cerebral sulcus, along the lateral surface of the parietal lobe to the longitudinal fissure, and then along the medial surface of the parietal lobe within the longitudinal fissure. ~ Receive nerve impulses for touch, proprioception (joint and muscle position), pain, itching, tickle and thermal sensation. 23Neurology Prepared by Group 433 (208 Primary visual area |- Located at the posterior tip of the occipital lobe mainly on the im (area 17) surface (next to the longitudinal fissure). Receive impulses the convey information for vision. _ Primary auditory |- Located at the superior part of the temporal lobe near the |: area (area 41 and cerebral sulcus, 42) It interprets the basic characteristics of sound such as pitch and rhy Primary gustatory |- Located at the base of the postcentral gyrus superior to the 1a area (area 43) cerebral sulcus in the parietal cortex. 7 . ~_Itreceives impulses for taste. Primary olfactory }- Located in the temporal lobe on the medial aspect. area (area 28) -_Itreceives impulses for smell. Centre of motor Motor output from the cerebral cortex flows mainly from the anterior part of each hemispls region in front of the central sulci. 1) Primary motor area (area 4) ~ Located in the precental gyrus of the frontal lobe. - Each region in the primary motor area controls voluntary contractions of specific mus or group of muscles. 2) Broca's speech area (area 44 and 45) ~ Located in the frontal lobe close to the lateral cerebral sulcus. ~ Speaking and understanding language are complex. activities that involve several sens association and motor areas. - Planning and producing if speech occurs in Broca’s speech-area. - From Broca’s speech area, nerve impulses pass to the: premotor regions:that control muscle of larynx, pharynx, and mouth. Association areas Consist of some motor and sensory areas, plus large areas on the lateral surfaces of the occipi parietal and temporal lobes and on the frontal lobes anterior to the motor areas. 1) Somatosensory association area (areas 5 and 7) ~ Is just posterior to and receives input from the primary somatosensory area, as wel thalamus and other parts of the brain. - Its role is to integrate and interpret sensations, eg. determine the shape anc object without looking at it. 2) Visual association area (areas 17 and 18) - Located in the occipital lobe. - It receives sensory impulses from the primary visual area and the thalamus. - It relates present and past visual experiences and is essential for recognizing and evalu" what is seen.Neurology Prepared by Group 433 (2007) 3) Auditory association area (area 22) ~ Located inferior and posterior to the primary auditory area in the temporal cortex. ~ Itallows one to realize whether a sound is speech, music or noise 4) Wernicke’s (posterior language) area (area 22 and possible areas 39 and 40) ~ Isa broad region in the temporal and parietal lobes. ~ This area interprets the meaning of speech by recognizing spoken words. - Itis active as you translate words into thought. 5) Common integrative area (area 5,7,39 and 40) ~ Is bordered by somatosensory, visual and auditory association areas. ~ It receives nerve impulses from these areas and from the primary gustatory area, primary olfactory area, the thalamus and parts of the brain stem ~ This arca integrates sensory interpretations from the association areas and impulses from other areas, allowing formation of thoughts based on a variety of sensory inputs. It then nsmits signals to other parts of the brain to cause the appropriate response to the ‘erpretation of the sensory signals. motor area (area 6) motor association area that is immediately anterior to the primary motor area. ‘Neurons in this area communicate with the primary motor cortex, the sensory association areas in the parietal lobe, the basal ganglia and the thalamus. A - Deals with learned motor activities of a complex and sequential nature. ~ Controls learned skilled movements and serves as a memory bank for such movement 7) Frontal eye field area (area 8) ~ In the fontal cortex is sometimes included in the premotor area. ~ It controls voluntary ‘scanning movements of the eyes ~ reading this sentence for instance. Mibfafc Abt fay, i Meas 25Send yn, BN hte Neurology Prepared by Group 433 (20 11) Frontal and temporal lobes. Anatomy, function and symptoms of lesion. Frontal Lobe [ (A) Brecentral gyrus | = Motor cortex: Supply contralateral movement of leg, face, ann - Localization of body part: + Leg: Supply partially in medial fissure surface + Am: Middle part * Face, tongue, jaw, lips: In inferior part of precentral gyrus. (B) Tractus Cortico-muscularis ~ Start from Betz éell in 5" layer of motor cortex, 1 upper motor neuron (UMN) of this tract + Pathology: Central (spastic) mono/hemiplegia (paresis) because UMN lesions depend on of damage, + Irritation: + Simple partial motor seizute/ partial seizure evolve to tonic-clonic convulsion + Movement involves contralateral face, limb * Jacksonian motor seizure (normal consciousness) * Localization seizure demonstrates site of tumor/other pathological process (C) Broca Area (Dominan) ~ Inferior part of dominant frontal lobe. ~ Expressive centre for speech, Pathology: Motor (expressive, Broca’s) aphasia/dysphasia Comprehension normal, speech * fluent, hesitation, repeated syllables poor handwriting Aphasia due to: Vascular disease, neoplasin, trauma, infection, degenetative disease. + Aphasia assess by: Spontaneous speech, naming object, repetition reading & writing, (D) Centre of graphia (Dominant) 7 = Middle of frontal gyrus - Pathology: Poor handwriting, associated with motor dysphasia (agraphia): (E) Supplementary Motor Area - a In front of motor cortex, superior of Broca area. ~ Centre of contralateral head & eyes turning, - Pathology: + Paralysis of head & eyes movement to opposite side + Head turns ‘toward’ pathological hemisphere, eyes look in the same direction, PRECENTRAL AREA ~ The vast part of the frontal Jobe anterior to the motor corten: pole of the frontal lobe, - For: personality, initiation, behavior ~ Pathology: Disinhibition, emotional lability Apathy Indifference Poor abstract thinking May akinesia, primi PARACENTRAL LOBULE = Middle surface, posterior part of superior frontal gyrus - For: Cortical inhibition of bladder & bowel voiding ~ Pathology: Incontinence of urine & faeces (loss of corti val inhibition) oly ve reflex (grasp, pout), ‘gait ay rasia’ (astasia-abasia), muscle resistanceNeurology Prepared by Group 433 (2007) Temporal Lobe (A) Wemicke’s language center ‘Some author describes gyrusangularis & wernicke’s zone together, because they situated very close in connecting part of 3 lobes (Parietal, Temporal in dominant hemisphere, Occipital) - Center of auditory, speech, comprehension. - Pathology: @ Wernick dysphasia (aphasia) Sensory/receptive © Comprehension impaired *. Unaware of language problem, use many neologism, nonexistent word, paraphrasia (repeated word) * Speech absurd, senseless (as a result of own speech comprehension disturbance) * Associated with impaired reading & handwriting (ii) Amnestic aphasia © name familiar object, but understand its purpose. Eg. Patient knows pen is for writing, {B) Auditory cortex Superior temporal gyrus, bilateral Cortical deafness may happen if Vbilateral lesion and very rarely (every cochlear nerve connect with both hemisphere) - Irritation: Auditory hallucination (C) Vestibular cortex ~~ Inferior temporal gyrus - Pathology: Cortical temporal ataxia, (final part of vestibular nerve) (D) Memory - Middle & inferior gyrus - Irritation: allucination with deja-vu/Jamais-vu Dejieva ~ “already seen” ~ Impression that something seen/ some situation: being experienced for Ist time has been __|___ previously seen/ experienced. Jamais-vu - Never seen - Subjective mental rxn being in a completely strange environment when in familiar surrounding. (E) Limbie cortex ~ Inferior & medial portion of temporal lobe: hippocampus & parahippocampal gycus. = Function: __. Olfactory zone __| = Bilateral = —_ Taste zone = Olfactory & taste fibres te minate in uncus * Disorder: only in case of vilateral lesions {| _ + Irritation: olfactory! tast'_ hallucination Center of emotion Pathology: (affective) behavior = Aggressive/ antisocie.| bohavior. Ll |_+_Inability to establist: new memories. Mit Cepfee AMMAR Ae ”Neurology Prepared by Group 433 (2007) 12) Parietal and occipital lobes. Anatomy, function and symptoms of lesion, ‘Anatomy Located on superolateral surface of | cerebrum Gyri © Postcentralis © Superior parietal -* Supramarginalis * Angularis Sule . Central (Rolando) Lateral (Sylvius) Interparictalis Parieto-occipitalis eee entral gyrus: © Main sensory cortex — receives afferent pathway of posture, touch, | passive movements. Supramarginal and angular gyri * Part of Wernicke’s language centre. © Integration of auditory and_ visual comprehension. Dominant hemisphere © Centers of calculation, handwriting and reading. Non-dominant hemisphere © Centre of appreciation of © Size, shape, texture and weight © Own body image © Own disease or defect © Geographical memory * Acquiring of skilled movement formed during life. ___Oecipi oA Located on posterior surface cerebrum Parts ‘* Cuneus (superior) © Lingualis (inferior) * Striate cortex © Parastriate cortex Sulei * Calearina ¢ Parieto-occipital *Primary visual reception) cortex (vis Parastriate cortex * Association visual cortex (relay i from: striate’ cortex. to parics temporal: and frontal lobes, on sat side and opposite side) —+ meaning visual image may be interpret remembered, ctc.Neurology Prepared by Gratip 433 (2007) [ Symptoms | Pasicentral vyrus | Cov ides i foflesion | ¢ Contralateral disturbances of contical /* Result of extensive bikuteral cortical i | sensation | lesion of striate cortex j ; © Postural sensation je Papillary light reflex normal without i © Sensation of passive movement conscious perception | © Accurate localization of light touch | ©Discrimination between one and | Aoton’s syndrome | _ tWo points jtlavolve both striate and parastriate | © Astereognasis cortices | oPerceptual — rivalry (sensory | « Affects interpretation of vision | inattention) + Pt unaware of visual loss | © levitation: i * Etiology © Simple sensory seizure | ” & Vaseuar disease (acksonian)/partial seize |S Hypoxia evolving 10 tonic-clonic seizure. © Hypertensive encephalopathy © Feel paraesthesia, tingling in) ¢ post tentorial herniation contratai. half of face, arm, ley acoording to localization of tumor or other pathological pre * Inability to direct voluntary gaze 4 1 * Associated with visual agnosia (loss of Supramarginal and angular gyri visual tecojmition) (7 |* Wemicke's dysphasia *Due to bilateral parieto~ occipital Dominant hemisphere issione | . Acalculia Visual hallucination © Agraphia *Elementary- unformed. appear as © Alexia patterns (zig-zag, flashes) i ; * Filled hemianopie Cield | I | Non-dominant hemisphere i | ¢ Autotopagnosia Visual illusion « Anosognosia * Objects appear smaller (micropsia) or * Geographical agnosia Jarger (macropsia) than reality. * Apraxia * Distortion of shape and color may © Dressing apraxia occur. © Constructional apraxia Prosopagnosia *Pt unable to remember names of | familiar faces * Associated with other disorders of ‘interpretation’ and naming with intact vision, *Due to bilateral lesions of occipito- temporal junction.Neurology Prepared by Group 13) Aphasia (disorder of speech). Wernicke's aphasia and Broca’s aphasia. To diagnosis, he cortical centers fur Cnguape)reside in the dominant hemisphere The donne hemis 's responsible for the cognitive Processing OF Tanguage, while the non-dominant hemisy produces and recognizes the emotional components of language part of thedfonial fol Grammatical structures and articulation Programs are represented in Broca’s area, wij sends its output to the motor cortex, 2) Wernicke’s area (receptive area: to understand Speech and give appropriate response) ~ Itis located at the posterior end of the Sylvian fissure, part of it at the angular gyrus of parietal lobe and part of it at the auditory area of the temporal lobe. ~ The angular gyrus processes auditory, visual, and tactile information, while Wemi area proper is the center for the understanding of language. 3) Arcuata fascicul is which link the two areas to integrate function, Etiology ~ Vascular disease - Neoplasm + Trauma ~ Infective disease ~ Degenerative disease Apha; - Impaired or absent com; prehension or production of, or communication by, speech, writing, Signs; due to an acquired lesion of the dominant cerebral hemisphere. ~ Syn: Logasthenia, dysphrasia, dysphasia, logagnosia, logamnesia, alogia, 3 Types of aphasia Broca’s aphasia (also called anterior, motor, or expressive aphasia) ~ Characterized by the absence or severe impairment of spontaneous speech, whl comprehension is only mildly impaired, ~ The patient can speak only with great effort, producing only faltering, non-fluent, and garbl words. Phonemic paraphasic errors are made, and sentences are of simple construction, ofte With isolated words that are not grammatically linked (agrammatism, “telegraphic” speech), ~ Naming, repetition, reading out loud, and writing are also impaired. LAfh (exe JWINeurology Prepared by Group 433 (2007) Wernicke’s aphasia (also called posterior, sensory, or receptive aphasia) ~ Characterized by severe(impairment of comprehension. - Spontaneous speech remains fluent and normally paced, but paragrammatism, paraphasia, and neologisms make the patient’s speech partially or totally incomprehensible (word salad, jargon aphasia). -—— . Naming fepetion of heard words))reading, and writing are markedly impaired, ea yet impaired Global apha: ~ Non-fluent speech & impaired comprehension. ~ Often associated with hemiplegia/hemianaesthesia & visual field deficit eas 83 ¢ yuduction aphasia Speech nonsensical but fluent (neologism & paraphrasia). Comprehension is normal, Repetition is poor. az clinical examination of Janguage includes Spontaneous speech - Naming of objects - Speech comprehension - Speech repetition, reading, and writing ‘The detaited assessment of aphasia requires the use of test instruments such as the Aachen aphasia test, perhaps in collaboration with neuropsychologists and speech therapists iy Re gizNeurology Prepared by Group 433 (20 14) Agnosia. Apraxia. Types. Topical Diagnasis. ye ‘Agnosia | Total or partial loss of the ability or recognize familiar objects or persons thr sensory stimuli as a result of organie brain damagy, The condition may affect a the senses and is classified accordingly as auditory, visual, olfactory, gustato: tactile agnosia. _ = ‘Apraxia | Impairment in th ity to perform leamed, skilled purposeful acts or to manip objects without sensory, motor and coordinating disorders. Specific types of agnosia that can be observed in impairment of parietal lobe function: Astereognosis Inability to distinguish between 2 various shapes or texture by touch, or inability to recos objects which are familiar by touch. Topical diagnosis: Lesions in the supramarginal gyrus of the contralateral Autotopagnosia * Inability to recognize or localize the various parts of the body because of organic brain dan * Symptoms & signs include disorientation of sides (left and right), finger agnosia, body par spatial neglect. Anosognosia + An abnormal condition characterized by a: real or feigned: inability to perceive a especially paralysis, on one side of the body. * Symptoms and signs include: denial of serious disease after informed, neglect of body which is paralysed. Geographical agnosia « Inability to recognize places and locations that are familiar to the patient. * Symptoms and signs include inability to get back to bed in the ward after toilet, loss in pati usual neighborhood. _ Apraxia _ - _ Ideational | Characterized by impairment caused by a loss of the perception of the use apraxia object. Motor ‘Characterized by an inability to use an object or perform a task without any lo: | apraxia perception of the use of the object or the goal of the task, ie cight Amnestic Characterized by an inability to perform the function because of an inal apraxi ‘Apraxia of | Is an articulatory disorder caused by brain damage and resulting in an inabilie speech program the position of speech muscles and the sequence of muscle mover" | necessary to produce understandable speech.Neurology Prepared by Group 433 (2007) Specific types of apraxia that can be observed in impairment of parietal lobe function | Dressing apraxia— Difficulty in dressing eg. Getting arm into shirt and buttoning, (©) Constructional apraxia — Unable to copy simple geometric pattern. SSS Topical diagnosis alana Lesions in the supramarginal and angular gyrus of non-dominant parietal lobe, Aculeulia~ Inability to count and perform simple mathematical manipulations. Agraphia - Loss of the ability to write resulting from injury to the motor language center. Alexia — Inability to comprehend written language. Also called word blindness. Topical diagnosis Lesions in the supramarginal and angular gyrus of the dominant parietal | ay ue Mest? a |yet [we Neurology Prepared by Group 433 ¢ 15) Corticospinal pyramidal tract. Symptom of lesion on different levels. nection: Control voluntary movement oo Pathway: Consists of 2 neurons - 1* neuron in precentral gyrus, its axons descent through all brain stem and decussate on border of medulla oblongata and spinal cord, then fibers go down in lateral funiculus late until the needed level.(Upper motor neuron) - 2" neuron lies in anterior horn and its axons exit spinal cord with anterior spiral root and go the muscle.(Lower motor neuron) NB: - Thalamus * involve in this system. - Nerve fiber crosses at the level of medulla oblongata. Pathology Limb weakness results from damage of gfotor system ftesion) at any level from motor cortex # muscle, Symptoms of Cl—-C4 | - Central tetrapalysis ad Anaesthesia below the lesion level ee | + Sphincter disorder (faccal and urine retention) C5-T2-|= Peripheral upper limbs palsy Central lower limbs palsy ~ Anaesthesia below the lesion level -_Sphincter disorder (faecal and urine retention) (T3-T12 |= Central lower limbs palsy. = Anaesthesia below the lesion level ___| + Sphineter disorder (faecal and urine retention) | = Peripheral lower limbs palsy - Anaesthesia below the lesion level a g = 5 a s & g a £ BS iB 8 2 g ze 8 fed g $3—S5 |~ Palsy is absent Sensory impairment in “saddle” area Incontinence of urine and faecals (detrusor paralysis) Lower Motor Neuron (LMN) Weakness (Please refer to Q17) ‘Upper Motor Neuron (UMN) Weakness (Please refer to Q16)Neurology Prepared by Group 433 (2007) 5 16) Upper motor neuron weakness. Clinical characteristics. Upper motor neuron (UMN) weakness ~ Central paresis ~ Results from decrease inhibitory funetion (Gon spinal cord'SC) > 1) Motor cortex lesion - 2) Brain stem lesion edo 3) Faniculus lateralis of SC lesion ober COYLE RIX xe wal signs Hypertonicity - SPASTISITY i Spastic paralysis. * Passive movement produces a ‘clasp-knife’. Doctor can feel the resistance only at the | | beginning of extension or flexion of limbs. | o Upper limbs — tone of flexors > extensors | © Lower limbs — tone of extensors > flexors | © Produce spastic posture “Wernicke-Mann” (arm and wrist flexed & leg extended), 2) ‘Hyperreflexia of deep reflexes (tendon, periostal) reflexs © | or absent of superficial reflexes. | 3) Pathological reflexes | ® Plantar response: (Babinsky’s reflex) — Stretch with a stick/a handle of hammer along the lateral side of sole towards to the big toe. * Rosalemma reflex > ymhmesia —Retlex happens at both sides wlicn lat at one side. ) Clonus Apply sudden and sustained flexion to fexicancistow po a few persistent beats (several Jerks), Ly thai veel by fertoniir’ 5 fas boty) Weal ibs Babies Pe pelagic | reflex ene 35Neurology Prepared by Group 433 (2007] 17) Lower motor neuron weakness. Clinical characte risties. = LMN weakness in: 1) Anterior horn lesion 2) Spinal root lesion 3) Plexus or peripheral nerve lesion - Peripheral paresis = Signs: 34, IF 1) Atonia ‘* Hypotonia with decreased resistance to passive stretch * Muscle tone is tested by passive movements of extremities 2) Atrophia © Muscle wasting * Wasting becomes evident within 2-3 weeks of onset 3) Areflexia * | or absent deep reflexes of ten¢on and periostal__ 4) Easciculation ‘© Involuntary contraction or twitching of muscle fibers under skin ¢ Observed in anterior horn cells lesion lowed §— eur? ffeopiee fb rophie: Avelenie fascicular’Neurology Prepared by Group 433 (2007) 18) Superficial sensory (spinothalamic) tract of pain, touch and temperature. Symptoms of lesion. ‘Tractus spinothalamicus The pathways of superficial sensation (pain, temperature, partly light touch). ~ Dendrites of the 1* neurons of this pathway get the information by skin receptors and carry it to the body cells of the /” neurons which lie in ganglions spinalis. axons enter the spinal cord and the second neurons lie in posterior horns. sxons of the 2" neurons cross the midline at the level of the segment, then rise in dus lateralis and end in contralateral thalamus opticus, where the 3” neurons lie. part of the pathways is called tractus spinothalamicus. The 4” neurons lie in gyrus entralis in the cortex, ‘Tractus Thalamocorticus Tractus Spinothalamicus Skin & toms of lesion ~ Seldom produce pain but usually a lack of awareness of pain & temperature. May results in’ * Tropic changes: Cold, blue extremities; hair toss; brittle nail © Painless buns * Joint deformation (Charcot’s joints) 37Neurology {Lesion of parietal | cortex Prepared by Group 433 Hemisensory loss of superficial sensation on contralateral side Thalamic lesion Pontine lesion ‘Ipsilateral to facial sensory loss * Loss of all modalities in the limbs (depending on the extent of the } Hemisensory loss of superficial sensation on contralateral side! Ayithout muzzle area sparing and a Jateral zaze palsy towards that | Medullary lesion ® Loss of all modalities in the limbs (depending on the extent of the © Hemisensory Joss of superficial sensation on contralateral side > without muzzle area sparing and a lateral gaze normal | tract lesion Partial cord lesion Dees = ikness of palate & tongue on side opposide to the limb sensory ‘Spinothalamie Loss of superficial sensation below a specific dermatome level (may Brown-Sequard Syndrome ree wn sacral sensation) on contralateral side © Loss of all modalities at one or several dermatome levels ipsilaterali * Loss of superficial sensation below a specific dermatome contralaterally * Loss of deep sensation up to similar level and limb weakness ipsilat Complete cord lesion Bilaterally loss of all modalities & leg weakness Central cord lesion @ Bilaterally loss of superficial sensation © Preservation of deep sensation _C/suspended’ sensory loss)Neurology Prepared by Group 433 (2007) 19) Deep sensory. Dorsal column pathways. Symptoms of lesion. - Proprioreception or deep sensation (perceived by proprioreceptors). - Proprioreceptors lie in muscle, joints, ligaments, periosteum and they provide information about body position and movement. -__Deep sensation is subdivided into: at | Vibration The tuning fork is used. [tis put ony prom: ty! sensation of radius and ankles. We ask the patient to compare the vibration on the is eyes cl asked about the direction of passive | tant phalanx (fingers) up and down, Joint sense Dorsal column pathway ¢ information (eg joint position) comes from joints by dendrites of 1“ neuron which lie in slion spinalis. \xons of the 1* neuron, avoiding posterior horn, pass to the funiculus posterior (white matter). ‘These fibers, form 2 pathways (Goll & Burdah columns), which rise to medulla oblongata where the 2 neuron (Goll & Burdah) lie (gracile and cuneate nuclei and columns). The fibers cross the midline right above nuclei and ascend to thalamus opticus where the 3“! nuclei lie. - Then the final part is tractus thalamocorticalis which connects nuclei with the 4" nuclei in gyrus postcentralis. Symptoms of lesions (Deep sensation) Produce a vliscriminatory type af sensory loss - Impaired 2 point discrimination - Astereognosis (failure to discriminate objects held in the hand) - Sensory ataxia (disturbed proprioception) Lesion of parietal | Hemisensory loss of deep sensation on contralateral side cortex ‘Thalamic lesion Hemisensory loss of deep sensation on contralateral side Pontine lesion Ipsilateral to facial sensory loss Loss of all modalities in the limbs (depending on the extent of the lesion) Hemisensory loss of deep sensation on contralateral side Loss of all modalities in the limbs (depending on the extent of the lesion) Hemisensory loss of deep sensatiowon-contralateral side + Weakness of palate & tongue on side opposide to the limb sensory deficit Brown-Sequard Syndrome Partial cord lesion | ¢ Loss ofall modalities at one or several dermatome levels ipsilateraily © Loss of superficial sensation below a specific dermatome level contralaterally ¢_Loss of deep sensation up to similar level and limb weakness ipsilaterally_ Complete cord | Bilaterally loss of all modalities & leg weakness \ lesion Central cord lesion | Bilaterally loss of superficial sensation © Preservation of deep sensation * (suspended? sensory loss) ‘Medullary lesion eeleeeNeurology Prepared by Group 433 20) Pathology of sensory system and its types. Sensory impairment may results in negative and positive symptoms : Negative symptoms ______|__ Positive | Superficial sensation Deep sensation |e Hyper ‘¢ Hypoesthesia © Sensory ataxia | © Pataesthesia © Dysasthesia * Pain * Anesthesia * Causalgia © Analgia * Thermalgia a | 'ypes of sensory impairment a) Peripheral. b) Central Peripheral type * Impairment of all kinds of sensation in zone of impaired nerve, plexus and spiral innervations. * Pattern of sensory deficit aids lesion localization. Examples: © Mononeuropathy — ulnar nerve © Plexopathy -- brachial plexus © Radiculopathy ~ spinal root LS Polyneuropathie type © “Socks and gloves” pattern, © Symmetrical distribution. © Affect both superficial and deep sensation due to multiple damages of periplieral nervy distal part of limbs. © Maybe accompanied by exogenic and endogenic neuropathies: a) Exogenic ~ Alcoholism, intoxication b) Endogenic ~ Diabetes, nutritional, hepatotoxic Conductive (central) type * Impairment of all sensations (superficial and deep) below level of damage, in brain or s cord. * Contralateral side of body and limbs; Ipsilatercl side of face Syringomyelitic type (dissociated © Damage of posterior horn of spinal cord. * Impairment of superficial sensation only since its pathway passed thru posterior horn of cord. © Loss of pain, touch and temperature sensations, © “Cape — like” distribution.Neurology Prepared by Gronp 433 007) 21) Control of the bladder and bowel function. Types of pathology. The urinary bladder stores (continence: kidneys. Parasympathetic fibers Arising in segments $2-S4 (sacral micturition center, detrusor nucleus) and traveling through the | pelvic plexus activate the detrusor muscle of the bladder, relax the internal sphincter (emptying). Chis leads to urination. and voids (micturition) the urine produced by the | Sympathetic fibers Arising from segments T10-L2 and traveling through the hypogastric plexus inhibit the detrusor (relaxation) and stimulate the internal sphincter contraction. This tends to decrease urination and promote fluid retention. Somatic motor impulses Arising from segments S2-S4 (Onuf’s nucleus) travel through the pudendal nerve to the external sphincter and the pelvic Tloor muscles. natosensory fibers © the bladder travel along the hypogastric and pelvic nerves to spinal levels T10-L2 and S2~ snveying information about the state of bladder stretch (overdistention is painful). tral nervous system (frontal lobes, b: nglia srves the voluntary inhibition of detrusor contraction. he pontine micturition center iggers the act of micturition, is under the influence of afferent impulses relating to the state of bladder stretch; its output passes to somatic motor neurons in the spinal cord that synergistically (innervate the detrusor and external sphincter muscles. Micturition Once a urine volume of 150-250ml has accumulated, stretch receptors generate impulses that pass to the pontine micturition center and also produce the sensation of bladder distention. Micturition begins when the dome of the bladder is stimulated to contract while the sphincter muscles relax. Contraction of the muscles of the abdominal wall increases the intravesical pressure and facilitates micturition. Site of Neurological Lesion and Type of Bladder Dysfunction 1, Supratentorial (frontal cortex, motor pathway) * Frequency +, urge +, urge incontinence, detiusor hyperteticxia y +, urge yb 2. Supratentorial and infratentorial ¢ Frequency +, urge +, imperative urinary urge, urge incontinence, detrusor hyperreflexia, impairment of voluntary voiding 4)Neurology Prepared by Group 433 (2 Spinal cord * Lesion above S2 (“reflex bladder”): hyper * Lesion of sacral micturition center (“autonomic bladder”): residual urine, detrusor are‘ impaired bladder reflex + Cauda equina, peripheral nerves: Residual urine, detrusor areflexia, impaired bladder impaired filling sensation, frequency + Note * Urge incontinence: Involuntary passage of urine on strong (imperative) urinary urge. * DSD: Detrusor-sphiicter dyssynergy. # Spinal shock with detrusor areflexia (bladder atony, “shock bladder”) and residual formation (overflow incontinence). The Bowel The uptake, transport, storage, and digestion of food, the absorption of nutrients, am elimination of waste matter are under the influence of both the extrinsic autonomic ner system and the intrinsic autonomic nervous system of the intestine. ‘The extrinsic system Modulates the function of the intrinsic enteric system in coordination with the function of organs of the body. * Brain stem nuclei: Subserve the gastrointestinal and enterocolic reflexes. * Cortical, limbic (hypothalamus, amygdala) and cerebellar centers (fastigial nu mediate the perception of satiety, the enteral response to hunger and odors, and em! influences on alimentary function. « The parasympathetic innervation (neurotransmitter: acetylcholine) of the esophagus, sto small intestine, and proximal portion of the large intestine is through the vagus nerve, whi of the distal portion of the large intestine and anal sphincter is derived from segments S: Parasympathetic activity stimulates intestinal motility (peristalsis) and glandular secretion. * Sympathetic innervation (transmitter: norepinephrine) is from the superior cervical ga to the upper esophagus, from the celiac ganglion to the lower esophagus and stomach, and! the superior and inferior mesenteric ganglia to the colon. Sympathetic activity in peristalsis, lowers intestinal blood flow, and constricts the sphincters of the gastrointestinal! (lower esophageal sphincter, pylorus, inner anal sphincter). The intrinsic system (enteric system Consists of the myenteric_and submucous ganglionic plexuses which are independent » networks of sensory and motor neurons and interneurons. The enteric autonomic nervous sy receives chemical, nociceptive, and mechanical stimuli, processes this neural input, and pre efferent impulses affecting gastrointestinal glandular secretion and smooth-muscle contracticNeurology Prepared by Group 433 (2007) Neurological causes of gastrointestinal dysfunction: GI Syndrome and Manifestati * Gastropa Delayed gastric emptying,nausea, vomiting, anorexia, bloating * Intestinal pseudoobstruction: Impaired intestinal motility, nausea, vomiting, bloating, weight loss, impairment of peristalsis * Constipation: Highly variable. Infrequent hard stools, straining, bloating, sensation of incomplete defecation, pain, flatulence, belching rvhea: Defecation rate +, liquid stools, tenesmus ‘ting: Gagging; yawning, nausea, hypersalivation, pale skin, outbreaks of sweating, | apathy, low blood pressure » Anal incontinence: Complete or partial loss of control of defecation 43Neurology Prepared by Group 433 (2 22) Optic Nerve. mptoms of Lesion, [ Optic nerve ‘yp Sensory _ Function | Visual acuity Main Pathways Retina — Optic nerve bundle (leaves eyeball behind optic disk) > Optic chiasm -~ Cont bundle to lateral geniculate bodies (makes connection with superior caliculli), ipsilateral to tuber externum of:pulvinar thamalii Both bundle ascends to posterior limb of inte capsule + Forms optic radiation -> Visual associated cortical areas (area 17, 18, 19 / cale sulcus). 3 different fibers and their pathway 1) Thalamus ~> lateral geniculate bodies —* posterior limb of internal capsule -> forms radiation and radiates in the dept of parietal lobe to visual associated areas. 2) Nucleuses of superior coliculli - tectospinal tract connection with CN III (nw pretectalis ~ Edinger-Westphal nucles). 3) Thalamus -> diencephalon (somatic and autonomic regulation eg. circardian cycle, v stress induced glucocorticoid secretion etc.), Histology Photoreceptor cells Rod-shaped dendrite (rods), con-shaped dendrite (cons) Pathway of light Cornea -- Aqueous humour -~ Pupil - Lens -- Aqueous vitreous -> Retina (inner li membrane) Main Coat of Eyeball ~ Comeoscleral coat (5 layers, covers 1/6 of cornea and 5/6 of sclera) - Vascular coat (includes choriod, iris and stroma of cilliary body) ~ Retinal coat Layers of retina 1) Pigmented epithelium 2) Layers of rod and cons 3) External limiting membrane 4) Outer nuclear layer 5) Outer plexiform layer 6) Inner nuclear layer 7) Inner plexiform layer 8) Ganglion cell layer 9) Optic nerve fibers 10) Inner limiting membraneNeurology Prepared by Group 433 (2007) Terminology Field of View (visual field Are physical objects and light sources in the external world that impinge the retina Inversion phenomena Image that reflected on the retina is inverted compared to actual object. ‘Normal field of view index Bae 35° to 70° (depends on size of nose) mporal_ | 90? — - ss 7 bs scorn ‘rior and | 50° to 70° (superior field of view depends on prominence of supraciliary arch) rior semiotics ypes of visual defects i) ual field defects 2) Colour vision disorders 3) Afferent papillary defects 4) Optic disk disorders Amaurosis | Total loss of vision in one or both eyes. Etiology is usually extraocular. Anopia | Loss of vision results from the absence or defects in one or both eyes. *_Hemianopia ~ Loss of vision in one half of visual field of both eyes. Homonymous | Visual loss on the same side of both eyes Eg. Left or right Heteronymous | Visual loss contralaterally of both eyes Eg. Bitemporal or binas * Quadrantanopia ~ Loss of vision in one quarter of visual field Eg. Superior temporal, superior nasal, inferior temporal and inferior nasal. * Altitudinal — Visual loss of the superior or inferior half of visual field (usually in cortical lesions). Amblyopia | Reduced visual acuity when ophthalmoscope examination appears to be normal. | | Scotoma A defect of vision in a defined area in one or both eyes. A common prodromal symptom is a shimmering film appearing as an island in the visual field which proceeds to focal visual loss. Disorders of Vision Classification 1) Monocular disorders 2) Binocular disorders Monocular Disorders ual field or acuity in 1 eye. ~ Common syndromes includes 45Neurology Prepared by Group 433 (2007 Ischemic Optic Neuropathy fugax) * Giant Cell (Temporal) Arteritis ~ Unilateral transient diminution or loss of vision (develops over seconds, maximum remains minutes and resolves over 10-15 minutes). - Embolisation is suspected to be the etiology. - TMB patients with coexist TIAs should be screen for atherosclerosis and carotid artery stenos Optic Neuritis + Inflammation of the optic nerve that produce the optic neuritis syndrome. ~ Etiology: Demyelinating diseases (most common), meningeal, parameningeal or intraocul inflammation associated with viral infection or post-viral syndrome. Rare causes include tox vitamin By, deficiency and neurosyphilis, ~ Symptom: Amblyopia occurs over hours to days, maximal within 1 week which associates with headact globe tenderness and ophthalmodynia. Ophthalmodynia typically exacerbated by movements, Central scotoma, papilledema (usual in retrobulbar neuritis), decreased i responsiveness. - Treatment: Treat underlying disease. Anterior Ischemic Optic Neuropathy (AINQ) - - Idiopathic infarction of the:anterior portiow of the optic-nerve: - Etiology: Usually considered as atherosclerotic origin. Occurs in old age (50) - Symptom: Sudden onset of painless Amblyopia (usually subtotal, maximal at onset and altitudinal papilloedema, peripapillary hemorrhage. Absence of papilledema and hemorthage, rap” expending intracranial mass and neoplastic meningitis should be 1/0 (ruled out). Giant Cell (Temporal) Arteritis = Progressive inflammatory disorder of cranial blood vessels, principally the temporal arters ‘Usual in women over 70 years old. - Symptom: Intractable headache, difficulty in chewing, weakness, rheumatic pains, and amaurosis if central retinal arter becomes occluded. Temporal artery may become pulseless, tender a swollen, but them maybe asymptomatic. = Disorders of visual field or acuity in both eyes. - Common syndromes includes: * Bilateral papilledema * Chiasmal lesions * Retrochiasmal lesionsNeurology Prepared by Group 433 (2007) Bilateral papilledema - Painless, passive swelling of the optic disk ~ Etiology: T ICP, congenital cyanotic heart disease, | CSF protein syndrome (eg, Guillain-Barré syndrome, spinal cord tumor etc.) ~ Indication for urgent evaluation to search for underlying pathology. Chiasmal Lesions = Usually pituitary tumors. Other causes include trauma, MS or other demyelinating diseases, berry aneurysm, metastatic or other infiltrating diseases. - Usually onset is chronic except in pituitary apoplexy. Retrochiasmal Lesions ~ There are 3 types of Retrochiasmal lesions: 1) Optic tract and lateral geniculate body lesions 2) Lesions in optic radiation 3) Cortical lesions 1) Optic tract lateral geniculate body lesions ~ Usually due to infarction or ICH. - Symptoms usually noncongruous homonymous hemianopia (can also be observed in thalamic lesions). 2) Lesions in optic radiation ~ Usually caused by cerebral insults. ~ Symptom usually congruous and. homonymous--with: acuity: completely normal in unaffected field. ~ Temporal lobe lesions may results in superior/inferior altitudinal quadrantanopia. - Parietal lobe lesions may results in homonymous hemianopia or ‘inferior altitudinal quadrantanopia, 3) Cortical Lesions ~ Various presentations. - May shows sparing of macular vision. Visual field defects and associated cortical lesions are shown below in diagram, 47Neurology Prepared by Group 433 (2 Cortical Lesion and Field of Vision R io A- Imaginary line continuous from Fissura Longitudinalis Cerebrii B Sulcus Calcarineus C- Gyrus Cuneus D- Gyrus Lingualis Types of cortical I ns and effect on field of SS ST — Superior Temporal SN — Superior Nasal IT - Inferior Temporal IN — Inferior NasalNeurology Prepared by Group 433 (2007) 23) Innervations of gaze. Oculomotor, trochlear and abducens nerves, Symptoms of lesion. [Function |~ Movement of levator palpebrae muscle - Movement of eyeball to superior lateral, superior, superior medial, medial and inferior medial direction -_ Accommodation and convergence due fo cont scle of sphincter pupillae_ Symptom of lesion in one side of otor nerve | Exophthalm iplopia Double vision due to disorder of accommodation and convergence (ipsilateral side) when looking superior lateral, superior, superior medial, medial and inferior medial direction -rgent squint Symptom of lesion in both side of oculomotor nerve - Ptosis of both eyes - Strabismus: Presence of squint CNIV___| Trochlear nerve ‘Type | Motor and vegetative Function | Innervate superior oblique muscle which contro! inferior lateral movement of eyeball. | 3 tom of lesion in one side of trochlear nerve - Diplopia: Double vision when looking downward (ipsilateral side) - Head may tilt to opposite side to minimize diplopia, CNVI Type Motor and vegetative Function | Innervate lateral rectus muscle which control lateral movement of eyeball Symptom of lesion of one side of abducens nerve - Diplopia: Horizontal, present only when looking to the paralysed side (ipsilateral side). + Convergent squint Etiology of lesion in CN II, IV and VI ~ Diabetic mononeuropathy - Tumor ~ Aneurysm - Infection ~ Intoxication 49see Neurology 24) Vestibulocochlear nerve. ympto.as of lesion. CN VIII — Vestibulocochlear nerve V2 parts: Auditory & Vestibular Prepared by Group 433 (2007) ‘Anatomy d nuclei in ic - Anterior, Posterior __| Medial, Lateral, Superior, Inferior Function | Cochlea converts sound waves into| Respond to rotational and line action potentials in cochlear neurons (J* | acceleration & along with a visual an order auditory neuron) and relay | proprioceptive input —_mainta information from spiral organ (of Corti) | equilibrium and body orientation i _) to the dorsal and ventral cochlear nuclei. | space. _ a Central * From the cochlear nucleus, 2” order | 1" order vestibular neuron Vie in th connection | neurons either pass upwards in the | vestibular division of the VII nerv lateral lemnicus to the ipsilateral inferior colliculus or decussate in the trapezoid body and pass in the lateral lemnicus to the contralateral inferior colliculus. © 3" order neuron from inferior colliculus on each side run to the medial geniculate body on both sides. + 4 order neuron pass through the internal capsule and. auditory radiation to the auditory cortex. and relay information from utricle, saccule and semiciucul canals to the vestibular nuclei (2 order neuron) which lie in midbrain. © 2" order neuron descend in th ipsilateral vestibulospinal tract. ¢ 2" order neuron connect wi oculomotor nuclei, cerebellum, cort (temporal lobe). Symptoms of lesion 1) Deafness Conductive » Causes: Otitis media, trauma. ilure of sound conduction to the cochlea Sensorineural | + Failure of action potential production or transmission due to disease cochlea, cochlear nerve or cochlear central conections . s: Infection(suppurative labyrinthitis, meningitis), trauma (petrow _____|__ temporal fracture), Meniere’s disease Cortical “TA bilateral or dominant posterior temporal lobe (auditory cortex) Z produces a failure to understand spoken language despite preserved heating 2 A sensai May be continuous/intermittent OR unilateral/bilateral OR high or low pitch. Causes: Cerebrovascular disease, anemia, hypertension. ph ulo ochleat fete nucleus ff vaste’ th ined! pevvor ft ngucl@h 1 of noise of ringing, buzzing, pulsing, hi: spire) organ IMO) to ssing, singing.Neurology Prepated by Group 433 (2007) 3) Sensory vertigo - Anillusion of rotatory movement due to disturbed orientation of the body in space. - The patient may sense that the environment is moving - May cause nausea & vomiting. - Causes: Disease of labyrinth, vestibular nerve or their central connections. 4) Damage to the vestibular apparatus results in an abnormality of stance and gait. The patient tends to fall towards the side of the lesion. Nystagmus is the sigh of the vestibular dysfunction Clinical examination (A) Cochlear Component 1) Weber’s test > Hold base of tuning fork (256 or 512 Hz) against the vertex. Ask the patient if sound is heard more loudly in one ear. i) Normal hearing Ui) Conductive deafness: Sound is louder in affected ear. (iii) Nerve deafness _: Sound is louder in the normal ear. 2 Rinne’s test Hold the base of a vibrating tuning fork against the mastoid bone. Ask the patient if note is heard. When note disappears- hold tuning fork near the external meatus. Patient should hear sound again since air conduction via the ossicles is better than bone conduction. - Conductive deafness: Bone conduction is better than air conduction: - Nerve-deafness _; Both bone and air conduction is impaired. (B) Vestibular Component 3) Caloric testing (vestibule-ocular reflex) Specific disorders of CN VIII system Meniere's disease is condition associated with endolymphatic hydrops involving the pars inferior of the otic capsule. Its cause is unknown. Symptom ~ The triad of episodic vertigo, tinnitus & fluctuating hearing loss. ~ Attacks last < 24hr - Occurs in clusters with weeks or month free, the remissions are longer. Treatment A low salt & administration of diuretics & betahistine (8-16mg, 31/d) 51 fy, SiNeurology Prepared bv Group Ee 25) Trigeminal nerve. Symptoms of lesion. CNV___|‘Trigeminalnerve = [Type Mixed CSCS Function | - Sensory nerve to skin and face -__Motor nerve to the jaw muscles Method - Lightly touch cornea with wisp or cotton, Access corneal reflex. ~ Measure Seanstion of light pain and touch across skin of face. - Palpate temporal & masseter muscles as patient clenches teeth, Nuclei (4); 1 motor 3 sensory © n. tractus spinalis (superficial sensation) * n, tractus mesencephalicus (deep sensation) n, sensorius principalis (vegetative) Anatomy Motor portion: Part of mandibular branch originates in the pons, passes through foramen ovale and terminai the mastication muscles. Sensory portion: Terminates in the pons. Consists of 3 branches: Ophthalmic (V1) = Contain sensory fibers from skin over the upper eyelid, eyeball; lacrimal gland, nasal c: nose, forchead and anterior half of scalp. - Passes through superior orbital fissure. Maxillary (V2) = Contain sensory fibers from nasal mucosa, hard palate, parts of pharynx, upper teeth, lip and lower eyelid. - Passes through foramen rotundum. Mandibular (V3) = Contains somatic sensory fibers (but not special sense of taste) from anterior 2/3 of tor lower teeth, skin over mandible, cheek (mucosa) and side of head in front of ear. = Passes through foramen ovale.Neurology Prepared by Group 433 (2007) ‘Symptoms of lesion ‘Trigeminal Neuralgia - Etiology unknown. - This condition affects mainly middle-aged and elderly people. - Trigeminal pain maybe symptomatic disorder which affect nerve root or its entry zone - Arterial vessel (aneurysm) compress trigeminal nerve root at entry zone — pons. Jemyelination ~ such a lesion in pons should be considered in young person with trigeminal scuralgia. - Paroxysms of sharp pain radiating into the territory of one or more of the trigeminal sensation divisions are characteristic. Pain is often set off by touching or washing the face, shaving, tooth brushing, eating, talking and exposure to cold. ~ The V2 and V3 divisions are usually affected first, but the condition may spread to involve all 3 divisions. ~ Paroxysms of pain last only a few seconds, - There are usually no abnormal motor or sensory signs of trigeminal nerve dysfunction, with the exception of finding localized trigger spots which set off pain when touched. Investigati - Cl scan & MRL Treatment - Membrane-stabilizing drugs (antiepileptics): * Carbamazepine 600-1200mg/day © Phenytoin. 53Neurology Prepared by Group 433 (2 26) Facial nerve, Symptoms of lesion. Bell’s palsy. [CN Vit] Facial nerve __ [Type | Mixed Function |- Facial expression -_Taste fibers sensation on anterior 2/3 of tongue Method and possible pathology - Ask patient: Wrinkle forehead (by looking upwards), close eyes, show teeth, and pe - cheeks. - Paralysis of the facial muscle (Bell’s palsy) Anatomy = Contains mainly motor fibers, but also parasympathetic fibers and taste fibers. Motor fibers Supply to the muscle of facial expression and stapedius musele. Parasympathetic | Supply to the salivary and lacrimal glands, visceral afferent fibers © fibers sensation of taste from the anterior 2/3 of tongue. _ + The motor nucleus lies in the lower pons. - The facial nerve exit from brain stem and cross the cerebellopontine angle. The nerve p through the facial canal in the petrous temporal bone with nerve intermedius and the together. - Facial nerve gives off several branches before exiting from the skull through the stylom foramen: 1. N. greater superficial petrosal: Controls of lacrimal gland. 2. N. stapedius: Supplies the stapedius muscle 3._N. chorda tympani: Carry taste fibres from the anterior 2/3 of the tongue ~The muscles in the lower face are controlled by the contralateral hemisphere,-whereas th the upper face receive control from the both hemisphere. Disorder of the facial nerve ~ In UMN (supranuclear) lesion, paralyses on the muscle in the lower half of the face on opposite side, flattening of contralateral nasolabial fold. - In LMN lesion, all the facial muscles are affected on that side (peripheral Bell’s palsy).Neurology Prepared by Group 433 (2007) Bell’s palsy Unilateral facial paralysis with sudden onset. Etiology - Viral infection eg. Varicella-zoaster, herpes simplex. - Bell’s palsy may be part of the syndrome of polyneuritis cranialis. - Ischemia and compression of facial nerve in the narrow canal of its course through the temporal bone. Symptoms = ‘Pain behind the ear may precede facial weakness Weakness develops within hours. Affected side becomes flat and expressionless. The palpebral fissure widens and the eyes not close. On the atlempting to close the eyes and show the teeth, the one eye not close and eyeball rotate upwards — Bells phenomenon, A proximal lesion may affected salivation, taste and lacrimation and maybe causes hypercusis. iagnosis Based on typical presentation. - Weakness of the entire half of the face distinguishes Bell’s palsy from supranuclear lesions (stroke, cerebral tumor), in which the weakness is partial, affecting the muscles in the lower part of face. ‘Treatment = Prednisolone given in high dosage in acute stage (40-60mg per day for 7 days) may reduce inflammation, - If for herpes simplex, give Acyclovir. Prognosis ‘Most patient recover in 4-6 weeks.Neurology Prepared by Group 433 27) Lower cranial nerves. Bulbar and pseudobulbar palsy. CN IX (Glossopharyngeal Ner This is mixed nerve with moror, sensory and parasympathetic function: Motor fiber Supply to stylopharyngeus muscle arise from nucleus ambiguus pa = __| medulla | Sensory fiber | Ionervate pharynx, carotid sinus, eustachian tube, an "pass to po: 3" of tongue(taste) and terminate centrally on the nucleus solitari Parasympathotic fiber | Arise in inferior salivatory nucleus and innervate the parotid glan Innervation (ipsilateral): Posterior 1/3 of tongue (taste) Soft palate and tonsils Constictuts of pharynx and parotid gland ‘The IX nerve emerges as 5 or 6 rootlets from the medulla, dorsal to the olivary nucleus passes with vagus and accessory nerves through the jugular foramen in the neck. Disorders of the glossopharyngeal nerve - Glossopharyngeal palsy from either medullary or nerve root lesions does not occs isolation. - When associated with X and XI CN lesions —>Jugular Foramen Syndrome. Glossopharyngeal Neuralgia > Short, sharp, lancinating attacks. of pain, identicalto trigeminal: neuralgia: in. natur affecting the posterior part of the pharynx or tonsillar area. - Pain radiates ear & triggered by swallowing: ~ Reflex: bradicardia & syncope: occur due-to stimulation of vagal nuclei: by discharges glossopharyngeal. - Treatment: Carbamazepine, microvaxcular decompression, section of LX nerve roots or CN X (Vagus Nerve] This is mixed nerve with motor, sensory and parasympathetic functions. Motor fiber Supply muscle of pharynx, larynx, soft palate arise in n. ambi (medulla), visceral muscle et, Sensory fiber Supply to larynx and pharynx, trachea and thoracic and abd viscera | Parasympathetic fiber | Arise in dorsal motor nucleus and supply bro1 muscle of vessels, GIT, kidneys. ‘The nerve exit from cranial cavity from jugular foramen. Symptoms of vagal damage ~ Are those relating to pharyngeal and Jaryngeal innervation, - Nuclear and vagal lesions, cause palatal, laryngeal, and pharyngeal paralysis.Neurology Prepared by Group 433 (2007) Disorder _ __ _ 7 Palatal | Unilateral | Minimal symptoms weakness [Bilateral ‘Nasal regurgitation of Muid, nas: ity of speech Pharyngeal | Unilateral | Pharyngeal wall droops on the ai . The uvula is pulled up weakness to opposite side on pronation and pharyngeal sensation is impaired |_on the affected side Bilateral | Marked dysphagia Laryngeal | Unilateral | Produce hoarseness of voice (dysphonia) with breathlessness and weakness stri Bilateral Stridor with breathlessness on exertion Bilateral palatopharyngeal paralysis Maybe occurs in motor diseases, bulbar poliomyelit diphtheritic neuropathy Examination of CN XI and CN X “hese nerves are considered jointly since they are examined together and their actions are “idom impaired. |.ook at palatal movement (ask patient to say ‘ah’ No palatal elevation on the affected side with the uvula pulled towards the normal side. 2) Check the gag reflex (CN XI-sensory, CN X- motor) - Depress patient tongue and touch palate, pharynx or tonsil on one side until the patient “gag” compares sensitivity on each side (CN IX). - Absence of symmetrical palatal contraction (CNX). - Absent gag reflex: loss of both sensation and motor power. 3) Note patient’s voice If those is vocal cord paresis (CN X palsy); voice maybe high pitch vocal cord examination is better leave to ENT specialist. 4) Note any swallowing difficulty or nasal regurgitation of fluids, CN XI (Accessory Nerve) = Is purely motor nerve. - Supply sternocleidomastoid (SCM) muscle and trapezius muscle. - Cranial portion of CNXI arises from nucleus ambiguus in medulla. = Spinal part arises in the ventral grey matter of upper 5 cervical segments, ascends along side of spinal cord and past through the foramen magnum. - Cranial and spinal path join together and form n.accessorius, it exits through the jugular foramen. - Spinal root provides motor supply to the trapezius and SCM muscle. - Unilateral lower motor neuron weakness produces a lower shoulder on the affected side (trapezius) and weakness in turning the head to opposite side (SCM), difficulty in lifting the arm above horizontal, in shrugging the shoulder. 57Neurology Prepared by Group 43 Clinical examination scm - Ask patient to rotate head against resistance, compare power and muscle bulk on each - Also compare each side with the patient pulling head forward against resistance. _N.B. The left SCM turns the head to the right and vice versa. Trapezius = Ask patient to shrug the shoulder and test against resistance, compare power on each si - Patient should manage to resist any effort to depress shoulders. CN XII (Hypoglossal Nerve) = Is purely motor nerve, supply the intrinsic muscles of tongue. - Nucleus lies in floor of IV ventricle and fibers pass ventrally to leave the brain stem late the pyramidal tract. - CN Xilexits through the hypoglossal canal and supply muscle on contralateral side. Clinical examination - Ask patient to open mouth, inspect tongue Eg, Atrophy (increase folds, wasting) Fibrillation (small wriggling movements) - Ask the patient to protrude tongue, note any difficulty or any deviation Eg. Protruded tongue deviates towards side of weakness Non-protruded tongue. cannot move to the opposite side - Disturbance of speech + Dysarthria, and dysphagia are minimal. Disorders - Lesion of CN XII results in atrophy and deviation of the tongue to the weak side. - Articulation is not affected. - Since each nucleus is bilaterally innervated, a unilateral supranuclear lesion * pr signs/symptoms. - Bilateral lesions — results in palsy of tongue. * Protrusion becomes impossible and articulation is disturbed. Generalized atrophy of tongueNeurology Prepared by Group 433 (2007) Bulbar Palsy ~ Lesion of CN IX, X, XII - Like peripheral paralysis Cause Symptoms __[ Clinical Examination * Tumor = Dysphonia ~ Gag reflex & jaw jerk — absent * Insult at MO - Dysphagia - Swallowing Trauma - Dysarthria | Encephalomeningitis ~ Regurgitation j - Local infection : | - Nasolalia (relaxation of soft i : palate) Je, monotone - Gag reflex absent - Hypoaesthesia - Tongue appeared folded - Fasciculation produce L writing appearance &seudobulbar Palsy Degeneration of corticobulbar pathway to CN IX,X,X1,XII motor nuclei on both sides. Like central paralysis Symptom Clinical examination M = All bulbar palsy symptoms ~ Nasolabial reflex sclerosis - Pathological subcortical reflex “2 ie. Marinascul Radovisha’ Many focal|- Hypereflexia lesions = Maybe +ve gag reflex (lacunar insult) |- Porst reflex - Grasp reflex - Palmomental reflex - Glabellar reflex ~ patient x blink in response to stimulus (ie. tapping between eyes) - Emotional disorder - Unprovoked outburst of laughing or “crying occurs Bulbar Palsy Pseudobulbar Palsy = Nerves or nucleus of CN IX.X, XI = Supranucleus fibres of CN TX.X.X1X1T - Lord sides + Clinical reflex present © Primitive reflex © Gag reflex = Emotional lability _ ssNeurology , Prepared by Group 4 28) Meninges of brain and spinal cord. Meningeal syndrome and raised intrac: pressure syndrome. Meninges have 3 layers of membranes: a) Dura mater b) Arachnoid mater ©) Pia mater Spaces between meninges: a) Epidural space ~ Between dura mater and skull b) Subdural space — Between dura mater and arachnoid mater ©) Subarachnoid space — Between arachnoid space and pia mater (CSF) Dura mater ~ Thick é& dense inelastic membrane - Most external of meninges |p bo ~ Encloses brain > cranial dura mater a - Encloses spinal cord > spinal dura mater Cranial dura mater cL Spinal dura mater 2 layers © Outer endosteal layer of cranial dura 1 1) Inner meningeal layer form 4 processes: becomes periosteum of vertebral column ¢ Falx cerebri ° Between spinal dura mater and end © Tentorium cerebelli layer > epidural space. © Falx cerebelli +» Contain: \ © Diagphragma sellae a) Loose connective tissues Also provide sheath for cranial nerves. b) Fat ¢) Venous plexuses 2) Outer endosteal layer — continuous with pericranium and orbital periostium. * Site of local anesthesia These 2 layers enclose venous sinuses that | drain blood from the brain, Arachnoid mater ~ Delicate membrane enveloping brain and medulla spinalis, ~ At base of brain, arachnoid mater form subarachnoid cistern by separating from pin mates - Subarachnoid cistern continuous below with spinal subarachnoid space and with 4" vents through foramen of Magendie. ~ Between arachnoid mater and pia mater —> subarachnoid space Subarachnoid space - Contains CSF ~ Cerebral subarachnoid space connects with ventricle of brain through 3 openines: Foran Magendie & 2 foramen of LuschkaNeurology Prepared by Group 433 (2007) jhe le { , Pia mater bee ~ “Vascular membrane” which knitted brain surface. ~ Contain arteries and veins. ~ Enter deep through cerebral gyri and form choroid plexus of ventricle. Meningeal syndrome L 1) Neck stiffness Neck, SUE DES. 2) Kernig’s sign Ker nicy 0 3) | aulzinski’s superior and inferior signs bondi u) Opisthotonos (in children) e 5) Associated neurological signs - eeicemt a. Impaired consciousness (90%) ee = b. Focal or generalized seizures (30%) ©. Cranial nerves signs (15%) d. Sensoneural deafness— due to direct cochlear involvement (20%) Raised intracranial pressure syndrome Etiology 1) Expanding mass~ Tumor, haematoma, abscess _ - 2) tbrain water content 3) tcerebral blood volume -- Vasodilation, venous outflow obstruction 4) tin CSF- Impaired absorption, excessive secretion Symptoms ~ Headache- Diffuse, at night or morning, worsen by stooping/ bending: ~ Vertigo ~ ~ Papilloedema- Pressure on optic nerve Oculomotor disorders~ Pressure on oculomotor and abducen nerves Vomiting - Neuronal damage results from brain shift —+ Tentorial or tonsillar herniation. Investigation: CT/MRI sean, ICP raonitoring ‘Treatment ~ Surgical removal of mass - Mannitol infusion - CSF withdrawal - Sedatives: Propofol, Barbiturates(thiopentone), Etomidate - Controlled hyperventilation - Decompressive craniectomy - Hypothermia ~ Steroids W ype wir 61Neurology Prepared by Group 433 29) CSF (physiology and pathology). Lumbar puncture, CSE ~ It is produced by the choroid plexus (located in lateral, 3" & 4" ventricles) and capillaries of the brain, ~~ ~ Circulation ¢ Lateral ventricle > Foramen of Monto — 3" ventricle —» Aqueduct of Sylvius ventricle > Foramen of Magendie and Lushka-> Subarachnoid spaces (SA). + Reabsorbed by the arachnoid bodies (Pacchionian granulations) ~ Average volume is 110m (20% in the ventricles and 80% in the SA. ~ Daily secretion is 500mL/day or 22mL/nr; 4-5 cyclesiday. SS etic Cami ah agit Functions solvent 1) Mechanical protection - A byateladbon & oer x 2) Maintain the constant ICP ~ 3) Removing of the waste products Analysis “4 a Pressure 100-150mmCSF (in horizontal position or 1 drop/sec) Colour Colourless and transparent _ | Protein 0.15-0.45g/L _ ~ Glucose 0.45-0.70g/L (~2.5-3.3mmol/L) which is about 40-60% of the blood (make sure to do the GTT to exclude DM); Leucocytes | 0-5 cell Erythrocytes | Absent 1) Ifall parameters are normal, RBC: 4-5 cells, if they are * Old cells > Hematoma * New cells > Trauma during the LP 2) When only protein t + Demyelination or tumour 3) When only glucose | + Hyperglycemia ~~ 4) Iflymphocytes} — Virus meningitis 5) Ifneutrophilst— Bacterial meningitis 6) Lymphocytes t and glucose | > TB menigitis stat mens i aren eesNeurology Po pared by Gray 433 2007) Lumbar puncture (LP) ~ Acquisition of CSF for analy ~ CSF drainage and pressure re ~ Technique * Correct position: Draw up the knees te the che: duction he head Ensure the back is cord i vel with the iliac crests) + Clean the area and inserts a few mL. of local anesthesia. + Insert the stylet of a 20G lumbar needle is filly home (2G for children’ + Insert at a slight angle towards the head. so that it is parallel i the spmneus Resistance is felt when the needle passes through the lig acum the dwa ind arachnoud layers, * Withdraw the stylet and collect the + PS: TUOHY needle allows insertion of ints instillation ior stimulationg electrodes pt epidural cansuls (eee St (for pain management) - When meningeal svndrome present. always de the L When meningeal syndrome present, always do the LE Contra-indication - Papilloedema (raised (CF) Skin disease at the lumbar a Anisoconia Bulbar syndromes Myocardial infarction + Edema ~ Brain tumor - Terminal stages of any disease ~ Platelet count <40.000 and prothrombin time <50% of control Contramdication = surbar~ punctuye. fl Matta sKin fisease. ot Phe (urbe Ares A lire. ' Mipcondial poferetion »eLerma + uleu : a Jumer Terininol stesye of oy? A Sees wea y Neurology Pe pred be Group 432 (20) 30) Autonomic Nervous Systent. Anatomy. functions and disorders A part of nervous system that egulates involuntery ital function. iy nding the acthaty af cardiac muscle. smooth muscle srcine yay —_ viscera). and glands ~ Ithas 2 main divisions, 1) Sympathetic nervous system 2) Parasympathetic nervous system Anatomy Autonon US SYS psutono { Suprasegmented p: into 2 par art (cent {. ©. Segmented part (peripheraly 9 Suinputh + Cerebral cortey. diencephaion erie | thinencephalon, reticular formation Uhoracolumbar ouiins * Controls segmented part 9 Poraxvnipatltric : - | Cron acial ou ~ Both with heir efferent and efferent pathways ~ Somatic nerve bundle that have high amount of autonomic fibers + Median nerve * Sciatic nerve © Femoral nerve ~ Thus lesions in these nerves cause. + Causalgia * Hyperemtia Anhydrosis or hypohydrosis Hyperesthesia dysasthesia Afferent sensory neuron Primary neuron consists of: 9 Primary visceral sensory neurons found in the peripheral ne n. Cranial sensory ganglia: the geniculate, petrosal and nodose ganglia, appended respectis! to cranial nerves VIL, IX and X. * These sensory neurons monitor the levels of COs, Os and sugar in the blood, arterial pressus and the chemical composition of the stomach and gut contentand alsa the gustatory sense * Primary sensory neurons s¥napse with telay visceral sensory ner oblongata. forming the nucleus of the solitary tract (nT information, * The nTS also receives input from a nearby chemosensory center. the area postrema. whi detects toxins in the blood and the CSF and is essential for chemically induced vomiting » conditional taste aversion rons located in the medulll which integrates all visces po enw pa aNeuroboy: : ‘ 4 : Sympathetic Nery ous System * Nucleus ~ Intermediolateral nucts1: ia ths hiteal hy a « cried theta Cota Ungal EAferent fibers forms sympathetic trunk (a pair of chains of venga stovhing sh ay ban sides of vertebral celumn from the bass of the ckull ts the casa oan 5 few intervertebral foramen Phers imme tiath afer enters the wrk petine or dakerierke “gud Gonape wal» tganuhoate fibeps on tg celine ganglio back te accompany sensory tiers Gusts ine reel g ind baie (oflictes) » Short pragang niglieans vibes * Supplies all sympathetic funetions to the whole seu: Parasympathetic Nerveny System * 2 main parts: Cranial outflow (CN HL VIL PS and 5 and gaced sath ov > Nucleus Accessory nucheus of CM HL Superior salivatory nucleus of Ci Uh Inferier salivatory nucleus of CY Dorsal vagal nucleus of CN Sacral parasympathetic nuclews- fi sactal outflow tite & * Elfferent-tibers run with appropriate eranial nerve or somati organs afitcat thers 4 * Cranial outflow supplies parasympathetic innervation from feud Ul teed of sigmoid vote whereas sacral outflow forms pudendal plesus and supplies rectus, sas eal on of pelvic cavity unetion _ Sympathe Syst __Parasy mpathetic Neryous System _ + Mediates “fight or flight” responds + Mediates “rest and digest” responds ‘sCentralization of “blood — vohime vin |» Vasodilation of the GL tact (postprandial vasoconstriction, > Bronchsconsirictio: + Enhance blood flow to muscles and lungs. * Miusis and spastn of accommodation + Bronchedilation — - ventilation and} = Stimulates salivary ghind x4 perfusion elerates peristalsis thus indirectly medi wes + Positive chronotropic and inotropic effzct, the absorption of nutrients thereby enhanced blood circulation to | + Inv olved in erection of genitals. via the pelvic peripheral vessels | splanchnic nerves (S:1) either caused by » Mydriasis and relaxes the lens. | reflex (tactile stimulation of erogenous zone! » Constriction of sphincter and { peristals | or psychogenic (ps»chologic or visual erotic + * Antierectile action and involves in emission | stimultion) phase of ejaculation of in male (hypogastric plexus). 0,Neurology Pr efetted by Group 43 Autonomic Nervous Svstem Disorders BP control + Orthostatic hypotension test ~ marked diep ia BEL 3. LO mmiigy > lesions + Cold presser test. stress test - cold and sttess do not raise BP — lesions > Valsalva maneuver © patient exhales aga‘nst closed ghotis and huld far 15 see intrathoracic pressure ~ | venous return ~ compensatory tachycardia), then brs normally ( f venous return -- overshoot BP vith bradycardia’ impaired responds lesions > Noradrenaline intusion | adrenaline in denerved smooth muscle: it marked rise BP following infuswon (hypersensitesity towar + lesions in postganglionic sympathetic fibers Heart Rate - Carotid massage (stinmuilites the carotid body - | parasyinpathetic efeets! Jivinishing the effect ~ lesions in either afferent or efferent pathway > Atropine test ~ no | in TEP and heart rate after infusion = losions in the sympotherie syster Sweating Induce sweating in patient and test with starch-iodide Paper Ge svesting = I sympathetic system ons in effere Pupillary Function - Check of light respends and accommodation. + Cocaine (stimulates adrenergic receptors) and atropine (blocks parasympathetic effect Mydriasis (if not appear ~ lesions in either system) Skin Temperature (coutrolled by sympathetic system > Acute pre or postganglionic lesions —> warm and hyperemic ~ Chronic postganglionic lesions -+ cold and eyanotic (denervation hypersensitivits towar catecholamine) General Disorders of ANS * Fever / pyrexia ~ | in both sympathetic and parasympathetic tone * Shock ~[in both sympathetic and parasympathetic tone Diencephalon Syndrome (hypothalamic syndrome ~ Also called as vegetative panic attack - Etiology: Part of PTSD. chronic stress, post syndrome. other chronic diseases. ~ Symptom’ Subfebrile temperature, tachycardia, tachypnea. diaphoresis, psychoemotiona! excitation, causalgic pain and may includes signs of endocrine disorders. Patien: usually experience increase amount of urine after attack - Treatment’ Treat underlying disorders. Symptomatic treatment includes tranquilizers. antidepressant, polyvitamine, and psychotherapy. operative complication, post-menopause-_ — Neurology Prepared by Group 433 (2007) ral Lesions LD) Complete or Partial Cord Lesions - Fecal retention (signs of UMN lesion). : oe [Male] Priapism up to 3 days — erection and ejaculation loss for months — rellex erection | { (tectile) appears but reflex ejaculation usually does not. Loss ot impaired fertility | (Female | Vaginal sensation and lubrication is loss. Fertility is preserved _ 2) Conus Lesions - Mixed UMN and LMN lesion signs. 3) Cauda Equina Lesions - Fecal incontinence (LMN lesion signs), - In beth conus and cauda equinal lesions, genital sensation is loss Loss ef ejaculation and erection reflexes and infertility are common in male. Specific Disorders of ANS 1) Idiopathic Orthostatic Hypotension Types * Sympathetic postganglionic neuron degeneration Shy-Drager Syndrome- Sympathetic preganglionic neuron (intermediolateral nucleus) / degeneration. (May have signs of extrapyramidal system disorders) Svinptoms Postural hypotension, anhidrosis with body temperature disorder, impotence, sphincter disturbance, pupillary abnormalities Differential Diagnosis with pharmacological test eg. noradrenaline infusion Treatment Fludrocortisone to t blood volume (monitor plasma potassium!), polyvitamine for neuronal regeneration 2) Diabetic Autonomic Neuropathy - Usual in patients with chronic DM type. | - Caused by destruction of nerve fibers in the autonomic system. - Appears as a constellation of disease eg. Diabetic gastroparesis. orthostatic hypotension, bladder dysfunction, impotence and retrograde ejaculation. vmptom Usually appears contradicting because involvement of both sympathetic and parasympathetic postganglionic fibers. Anemnesis morbi and vitae in this case is important. Treatment Treat as diabetic patient eg. Blood glucose level control e.t.c. 67rds) A Neurology Prepared by Group 433 (20¢ SPECIAL PART 31) Lower back pain, lumbar radiculopathies, prolapse of lumbar vertebral dise. Actiolo pathogenesis, clinical features. Lower back pain due to lumbar radiculopathy and profapse of lumbar vertebral dise Lumbar radiculopathy Irritation or affection of spinal nerve root (mainly LS and $1) - Etiology : slopped/ herniated/ rupture dise ~ Pathogenesis * Rupture due to strenuous physical activity, congenital defect. injury *Gelatinous material leak out of disc and compression of the root of nerve caus inflammation of root surface . Clinical features wer back pain - Begin abruptly - Sharp and severe, travel quickly down back of leg, ankle and feet - May intensified during coughing, sneezing and sitting” + Relief when draw the knee close to the chest When irritation of level of = L3-L4: Pain through thigh, muscle weakness and numbness in shin and ankle. ~ L4-L5: Pain through back of thigh and calf muscle, muscle weakness along dorsal part of foot and big toe. ~ L5-S1: Pain in calf. numbness in foot, weakness of ankle reflex. Lumbar disc prolapse Etiglogy Acute dise prolapse * Herniation © Posterolaterally compress the adjacent root wast! © Centrally compress root within cauda equina * Hypertrophy of degenerated facet joints Pathogenesis: Compression of root of nerveNeurology Prepared by Group 433 (2007) ~ Injury: A history of falling/lifting heavy weights often precedes the onset of symptoms. - Leg pain - Paraestliesia: Numbness and tingling in affected zone Mechanical sign Restricted spinal movement Scoliosis, normal lumbar lordosis is lost wight leg raising: LS & SI root compression causes limitation to <60° from the horizontal and produces pain down the back of the lex. Reverse leg raising (femoral stretch): Test for irritation of higher nerve roots (L4 and above) swological deficit: Depends on the predominant root involved © La: Quadriceps wasting and weakness, sensory impairment over medial calf and impaired knee Hl jerk, ,* LS: Wasting and weakness of dorsiflexors of foot, extensor digitorum longus and extensor | hallucis longus, wasting of extensor digitorum brevis, sensory impairment over lateral calf and dorsum of foot # SI: Wasting and weakness of plantar flexors, sensory impairment over lateral aspect of foot and sole, impairment of knee jerk. Central disc protrusion ~ Sign and symptom are bilateral (although 1 side is often worse than the other) ~ Leg pain: Extends bilateral down back of the thighs ; may disappearwith’the onset of paralysis - Paraesthesia - Sphincter paralysis: Retention of urine, impaired anal sensation, constipation ~ Sensory loss: Extends over all or part of sacral area (saddle anaesthesia) - Motor loss: Present as foot drop with complete loss of power in dorsiflexors and plantarflexors of both feet = Reflex loss: Ankle jerks absent 69crsar 4 7700 Neurology Prepared by Group 433 (20! 32) Bell’s palsy. Etiology, pathogenesis, clinical features, treatment. Definition ~ Acute paralysis of face because of inflammation/ischemin/compre: within facial canal or at the stylomastoid foramen, — ~ Usually unilateral on of facial nerve (CN Etiology ~ Viral infection eg, Herpes simplex virus, varicella zoster virus ~ May be part of the syndrome of polyneuritis cranialis Pathogenesis ~ The cause of idiopathic Bell's palsy is felt to be an infection of the nerve by a virus or bac with the facial muscle becoming cut off from its nerve supply. - The patient then develops a secondary muscle paralysis (palsy) of the facial muscles, = The muscle paralysis is caused by inflammation and nerve damage (autoimm demyelination) instead of a cut off of the blood supply to the nerve (ischemic compression = Sudden onset of paralysis or weakness on one side of the face, making it difficult to sm close eye on aflected side. - The palpebral fissure widens, and the eye close. On attempting to close the eyes & show teeth, the one eye Electromyography shows multifocal demyelination showing of motor conduction Treatment There's no cure for GBS. However, certain therapies can lessen the severity of the illness accelerate recovery for most people. 2 main treatments a) Plasmapheresis - also known as plasma exchange * Isa type of "blood cleansing" in which damaging antibodies are removed from the bs * Consists of removing the liquid portion of the blood (plasma) and separating it {rom actual blood celis. The blood cells are then put back into the body, which manuf more plasma to make up for what was removed. b) Intravenous immunoglobulin (IVIg) * Immunoglobulin contains healthy antibodies:from blood donors: © High doses of immunoglobulin can:block the damaging, antibodies in the blood that contribute to GBS. ~ Based on supportive treatment to prevent respiratory and autonomic complication * Early tracheostomy or intubation is indicated if there is progressive bulbar and weakness, * Hypotension from dysautonomia: IV volume infusion and the use of vasopressor age * Extreme hypertension: short-acting and titratable antihypertensive medications, suck IV labetalol. * SC low molecular weight heparin to prevent thromboembolism for those immobility. ~ Pain medications (analgesic drugs) including acetaminophen and NSAID, possib' combination with narcotic pain-killers. - Physical therapy - help regain strength and proper movement. - Whirlpool therapy (hydrotherapy) may help relieve pain and retrain the movement affected limbs.Neurology Prepared by Group 433 (2007) 35) Upper limb mononeuropathies. Etiology, pathogenesis, clinical features and treatment. Long Thoracic Nerve ~ Carrying heavy objects ~ Winging of the scapula when (CSC6C7) ~ Strapping the shoulder arms are stretched in front ~ Limited brachial neuritis - Diabetes Mellitus anterior muscle Nerve - + of abduction of ~ Strapping the Shoulder - Limited brachial neuritis ~ Weakness of external rotation - Diabetes Mellitus of arm (infraspinatus) figoaepinatss & infraspinatus muscles | . oe _ [Axillary Nerve (posterior | - Shoulder dislocation - Weakness of abduction of cord\(C5C6) - Limited brachial neuritis shoulder b/v 15-90° and sensory loss over the outer Supplies aspect of the shoulder |. Deltoid & teres minor muscles ee = Musculocutaneous Nerve | - Fracture of the humerus - Weakness of elbow flexion (lateral cord) (C5C6) = Systemic causes and forearm supination with characteristic sensory loss and Sensory Supplies absent biceps reflex - Lateral border of arm Radiat Nerve (Posterior cord) (C6C7C8) / Function : Extension (eg. ‘hi’, ‘good ‘Sensory = Dorsum of hand \| supply - The nerve descends from the axilla, winding posteriorly around the humerus. i The deep branch — the posterior interosseous nerve — lies in the posterior compartment of the forearm behind the interosseous membrane Damaged by |- Fractures of the humerus Prolonged pressure (Saturday night palsy) IM injection Lipoma, fibroma or neuroma Systemic causes Results in | - Weakness and wasting of muscles supplied, characterized by wrist drop with flexed fingers (weak extensors) - Sensory loss on dorsum of hand and forearm : - Loss of triceps reflex (when lesion lies in the axilla) and supinator reflex = The posterior interosseous branch of the radial nerve can be compressed at its point of entry into the supinator muscle. - The clinical picture is similar to a radial nerve palsy, only brachioradialis and wrist extensors are spared - Examination shows weakness of finger extension with little or no wrist drop. 75Neurology Prepared by Group 433 (2 Median Nerve (Lateral & medial cords) (C7C8) Function; apposition of thumb, ‘hand like monkey’ Sensory = Palmar surfaces of the radial border of the hand supply - ‘The nerve lies close to the brachial artery in the upper arm. It passes unter i _| transverse carpal ligament as i alm of the hand Damaged by |- Injury in axilla eg. Dislocation of cater compression in the fore anterior interosseous branch, compression at the wrist (carpal el syndrome Resultsin {- Weakness of abduction and apposition of thumb = Weakness of pronation of the forearm Deviation of wrist to ulnar side on wrist flexion Weakness of flexion of distal phalanx of thumb and index finger Wasting of thenar muscles is evident Sensory loss is variable but most marked on index and middle fingers _ Carpal tunnel syndrome ‘The most common entrapment neuropathy, more frequent in women, results from median entrapment under the transverse carpal ligament at the wrist. Causes - Connective tissue thickening - Rheumatoid arthritis - Acromegaly - Hypothyroidism - Infiltration of ligament eg. Amyloid disease - Fluid retention eg. In pregnancy, weight gain Symptoms ~ Pain, especially at night, and paraesthesia, eased by shaking the hand or dangling it out bed - Objective findings may follow with cutaneous sensory loss & wasting & weakness of th muscles (abductor & opponens pollicis). Percussion on the nerve at the wrist pro heightened paraesthesia (Tinel’s sign) - Nerve conduction studies are helpful in confirming diagnosis by showing slowing of cond over the wrist ‘Treatment = Of the cause, weight loss and diuretics - Surgical division of the transverse ligament if symptom fail to improve produces exc results (90% symptom free)Neurology LF Sensory supply Ulnar Nerve ( Prepared by Group 433 (2007) edial cord) (C7C8) ion (eg. ‘bye-bye) _ - - Both palmar and dorsal su of the ulnar border of the ha ~ In the upper arm the nerve is closely related to the brachial artery and the median nerve, and passes behind the medial epicondyle of the humerus into the forearm ~ In the hand, close to the hamate bone, it divided into deep and superficial branches _—_ ~“injury at elbow, eg, Dislocation | - Entrapment at elbow or distal to the medial epicondyle = Pressure on the nerve in the palm of the hand damages the deep branch | resulting in wasting and weakne: - Weakness and wasting of muscles supplied, with a characteristic posture 0 hand — wnar claw hand - As well as sensory loss. The level of the lesion dictates the extent of the motor paralysis. Nerve conduction studies are helpful in confirming entrapment at the elbow - Surgical transposition may be necessary in such cases 7Neurology Prepared by Group 4 36) Lower limb mononeuropathies. Etiology. pathogenesis, clinical features and (reatm | Definition noneuropathy _ a stiopathogenesis | Systemic Damage (o a nerve secoudary to other illnesses linesses _| Eg: DM, sarcoidosis, vasculitis, leprosy Entrapment | ‘Damage to a nerve when it passes through a tight space __...| Bg: Actomegaly, myedema, pregnancy - Trauma Damage of nerves and surrounding tissues _| Ex: Fractures, tumor, abscess Affected nerves and their clinical features 1a —_—} Damaged by | mesults in. Femoral Nerve ~ Fracture of upper femur ~ Weakness (L1L213) = Congenital dislocation of hip, hip | - Weakness of knee extension surgery wasting of thigh muscle + Neoplastic infiltration + Sensory loss over anterior & » ~ Psoas muscle abscess of thigh - Reccieioan int iopsoas muscle | - Loss knee jerk Sane process as femoral n, - Weakness of hip external com During fabor and adduction Compression by hernia of |- Sensory loss over inne: ator canal ~__Loss adductor reflex gs or traumatic ~ Weakness of hamstring is (L4L5S182) dislocation with loss of knee flexion + Penetrating injuries ~ Weakness of distal log and + Misplaced IM injection muscles + Entrapment at sciatic notch = Sensory loss over outer leg _ __| = Systemic causes ______| =_Loss ankle reflex Common ~~ Peroneal | - Trauma to head of fibula = Weakness of dorsiflexion Nerve (L4L5) > Pressure from kneeling, crossing | eversion of foot legs ~ Walks with a “foot drop” ~ Systemic causes + Sensory toss over dorsum a __ouler aspect of foot _ Posteror Tibial Nerve | - Trauma in popliteal fossa = Weakness of plantar (S182) > Fracture of tibia inversion of foot ~ Systemic causes = Cannot stand on toes = Sensory loss over sole of foot = Loss ankle reflex _ Tarsal tunnel syndrome ~ The posterior tibial n. may be entrapped below the medial m —burning pain in the sole of foot | : = _ Surgical decompression is often required : Plantar and Small | - Compression of these nerves at] - Localized buming + Anterdigital Nerves sole of foct of foot }- Pain and analgesia in ac bo . _____|__halves of neighboring toes _Neurology Prepared by Group 433 (2007) Treatment 1) Regime: Bed rest 2) Analgesics: Analgin, ibuprofen 3) Anti-inflammatory: Prednisolon 4) Physiotherapy *7) Poliomyelitis. Aetiology, pathogenesis, clinical features, prophylaxis and treatment. ~ Its an acute viral infection (potiovirus)in which the:anterior:horn cells of the spinal cord and motor nuclei of the brain are selectively involved. ~ It isa major cause of paralysis, Pathogenesis ~ Spread by the faecal oral route. Once ingested the virus multiplies in the nasopharynx and GIT. ~ Penetration of GIT results in viraemia but CNS involvement occurs in very small proportion. Most infected patients are asymptomatic. ~ Virus excretion continues in the faeces for as Jong as 3 months after the initial infection (carrier state), ~ Initially, inflammatory meningeal changes. Followed by inflammatory cells infiltration (polymorphs and lymphocytes) around the brain stem nuclei and anterior horn celle ~ Neurons may undergo necrosis or central chromatolysis - Microglial proliferation follows. Infection may result in 1. Subclinical course + resultant immunity (majority) 2. Mild non-specific symptoms of viraemia + resultant immunity 3. Meningism without paralysis (preparalytic) + resultant immunity 4. Meningism followed by paralysis (paralytic) + resultant immunity 79Neurology Prepared by Group 433 (200 PREPARALYTIC stage General symptoms: Fever, sweating, malaise, headache, mild GIT upset Progres: Specific symptoms: Severe headache, back and limb pains, muscle tenderne: of meuingism, Progresses Improves PARALYTIC stage Improves Spinal form + Muscles fasciculation, muscle pain worsen ~ Paralysis develops. (widespread or localized, ascending or descending, max 24hrs afler the ouset of this stage) ~ May involve respitatory muscles Bulbar for ~ Pharyngeal, laryngeal, lingual and facial weakness, > May involve cardiac and respiratory muscles, A mixed form can occur — Bulbospinal form gnosis ~ During meningeal phase, consider other causes of acute meningitis. ~ Once paralytic phase ensues, distinguish from Guillain-Barre syndrome (} CSF protein with: tin cells) & transverse myelitis. ~ The clinical pictures ~ CSF examination: Polymorphs and lymphocytes [, protein t with normal glucose ~ Serological test ~ Virus isolation Differential Diagnosi - Acute meningitis ~ Guillain-Barre Syndrome - Transverse myelitisNeurology Prepared by Group 433 (2007) Treatment Bed rest and Suid balance carefully maintained Respiratory failure required ventilation Avoid the development of deformities in affected limbs with physiotherapy and splinting, Antiviral preparation Prophylaxis Lceination 1) Inactivated (Salk) Polio vaccine (IPV. Formalin inactivated virus. 2 injections, } month apart, are followed by booster at 6 months; this prevent CNS invasion, but does not stop viraemia. 2) Qral (Sabin) Polio vaccine (OPV) (vaccine of choice) Live attenuated virus given orally and will simulate subclinical infection, 3 doses 2 months apart, - In epidemics, a mortality of 25% results from the respiratory paralysis. Improvement in muscle power usually commences one week after the onset of paralysis and continues for up a year, Only a proportion of muscles remain permanently paralysed; in these, fasciculation may persist. In affected limbs, bone growth becomes retard with shortening as well as thinning. Post-Polio Syndrome * A significant disproportion. of polio patients: develop late: sequelae-often:30-years’ after initial illness - fatigue, myalgia and new weakness are characteristic. 81Neurology Prepared by Group 433 (2 38) Acute Myclitis. Etiology, pathogenesis, clinical features, treatment. ~ Sudden onset severe inflammation of the spinal cord with associated motor, sensory autonomic dysfunctions. Classification 1) Transverse 2) Longitudinal 3) Diffused (ascending) Etiology 1) Infection ; Retrovirus ete 2 Spp. ic eg. Schistosoma spp. rug abuse, medications Immune Mediated ~ Lead, arsenic, mercury ligh dose p} trauma Acute Transverse Myelitis Single or multiple complete segmental acute inflammation of gray and white matter of the sp" cord characterized by acute/subacute segmental demyelination or necrosis. Clinical Features ~ Pain syndrome —» headache, back pain, neck pain. - Fever and meningeal symptoms may predominant in infectious etiology. ~ Band like tightness around the chest or abdomen —» radicular pain ~ Altered level of consciousness. - Bilateral paresis, hypoesthesia/anesthesia below level of lesion, deep sensation maybe ret «for 2. to 3 days more. Z ~ Fecal and urinary retention developed over hours to a few days. ~ Progressive neurological deficits to complete transverse sensor motor myelopathy. ~ Symptoms depend on level of lesions. Diagnostics 1) History— Suggest etiologic factor 2) Physical examination—> Level of lesion 3) Laboratory findings ° Blood Tests - Leukocytosis - TESR ~ Detectable abnormal level of heavy metal © CSF analysis—Depends on etiology 4) Conformation of diagnosis Full spinal X-ray and MRI - Focal cord sign changes - Cord enlargementNeurology Prepared by Group 433 (2007) Management ~ Hospitalization and bed regimen. ~ Paraenteral nutrition in acute phase and hypoalergic diet in recovery phase. ~ Catheterization to relieve urinary retention. In acute phase ee — Supportive treatment | ® Analgesics | Detoxification therapy » Mechanical ventilation for respiratory paralyzed patients (lesions about C3) © Splints or immobilization equipments for patients with spinal shock syndrome Hiologic treatment | Depends on the etiology eg, Antiviral, antibiotics, antifungal, antiprotozoal agents, chalating agents, high dose ghicocorticoids or in severe autoimmune state cytostatics and plasmapharesis can be prescribed, © Continue supportive therapy if needed ¢ Follow up etiologic therapy * Vitamin supplements * Physiotherapy Constant nursing to avoid bed sores ~ Recovery may extend from duration of 2 to'12 weeks to 2 years: - Usually there will be no recovery if no signs of recovery of neurological functions are observed during the first 3“ to 6" months: 83Neurology Prepared by Group 433 (2009 39) Acute bacterial meningitis. Actiology, pathogenesis, clinical features, treatment. - Inflammation of meningeal which caused by bacteria agent ~ It is seoondary, acute or subacute, pus (septic) meningitis Etiology ~ Meningococeus (Neisseria meningitidis) ~ Pheumococcus (Streptococcus pneumoniae) ~ Haemophilus influenzae ~ Listeria monocytogenes ~ Streptococcus pyogenes - Staphylococcus aureus Pathogenesis stly appear different bacterial infection symptoms, such as sore throat, tonsillitis, pnew otitis and ete, Later inflammation spread to meninges by crossing the blood brain barrier, The presence of BBB limits host defence mechanism and enables multiplication of organise A purulent exudate most evident in basal cisterns extends throughout the subarachnoid ¢ - The underlying brain, although not invaded by bacteria become congested, edematous ischemic, Integrity of pia mater normally protects against brain abscess formation. ~ Inflammatory exudates may affect vascular structures crossing the _ subarachnoid space, to arteritis or venous thrombophlebitis with infarction. - Cranial nerve may suffer direct damage. - Hydrocephalus can result from obstruction of CSF flow in the ventricles and subara: space. Clinical features - Classical clinical triad: Fever, headache (diffuse) & neck stiffness. - Intoxication symptom: © Varies and depend on agent * Eg. A respiratory infection, otitis media/pneumonia asso. with muscle pain, back: lethargy, Tfever, nausea, vomiting, confusion ~ Meningeal symptom: Neck stiffness, Kernig’s sign, Brudinski - Absent of focal neurological signs. - In infant and small children: * Opisthotonos (severe neck, back and limb stiffness, ultimately resulting in chara arched posture) and “bulging frontanelles”. Teer Co Complication Tye ~ Severe frontal/ occipital headache, 1 gradually - Photophobia, phonophobia - General hypersensitivity - Nausea, vomiting sign plassificotion of muibiple SeleveoSNeurology Prepared by Group 433 (2007) Associated neurological sign ~ Impairment of consciousness (90%) - Focal or generalized seizures (30%) ~ Cranial nerve sign (15%) - Sensorineural deafness ( due to concurrent otitis media, but to direct cochlear involvement - 20%) Laboratory diagn: ~ Lumbar puncture - CT scan ~ CSF examination - Serological/immunological test + Blood culture = Check serum electrolytes - Detect the source of infection CSE_analysis ~ Pressure: t - Color: Yellow/ green ~ Cytosis: Neutrophil (around 1000-2000) - Protein: 1/ normal = Glucose: |/ normal ‘Treatment Initial therapy (before organismidentification) = Children ( Through nasopharynx go into blood stream and cause septicemia ~ Primarily infects children and has very acute onset; usually dusing winter/spring Clinical features Meningeal syndrome ~ Intoxication symptom: nausea, vomiting, tachycardia, dypsnea - High fever accompanied with loss of consciousness ~ Athralgia ~ DIC syndrome -> hemorrhage and purpuric skin rash ~ Focal neurological symptom not typical Diagnosis 1) CT scan to exclude intracranial mass i 2) Blood culture and distinguish antibiotic resistance (Ad 3) Lumbar puncture and laboratory diagnosis of CSF ey - Milk-like color = T pressure N = } neutrophils Sar ~ { proteins = Glucose is | or normal 4) Immunological tests (ie. Luminescence assay, PCR) 5) Detect the source af infection (ie. X- ray of sinus) ‘Treatment 1) Before the causative agent has been identified, introduce penicillin G or cephalosporin, 2) Dexamethasone 10 mg every 6 hours for 4 days, 3) After identification - Antibiotic; + Benzylpenicillin 180mg/kg/day in children and 20 million units for adult. * Alternative — Chloramphenicol, cetatamin 4) Analgetics 5) PiasmapheresisNeurology Prepaved by Group 433 (2007) 41) Tuberculous meningi treatment. logy, pathogenesis, clinieal features, prophylaxis and ‘Tuberculous Meningitis (TBM) = Is the commonest manifi infection of M. tubercul = In children, it usually results from bacteraemia following the initial phase of primary oulmonaty tuberculosis in adult, it may occur many years ater the primary infection tion of tuberculous infection of the nei ous system caused by the Etio-pathogenesis Following bacteraemia, metastatic foci of infection (granuloma, tuberculoma) lodge in: 1, Meninges 2, Cerebral or spinal tissue 3. Choroid plexus Rupture of these encapsulated foci results in spread of infection into the subarachnoid space sseous necrosis). In adult, reactivity of metastatic foci may occur spontaneously or result impaired immunity (eg. recent measles, alcohol abuse, steroids). “(he clinical feature result from: * infection ¢ Exudation ~ May obstruct basal cistem & result in hydrocephalus © Vasculitis ~ Secondary to inflammation arounf vessels & results in-infarction of brain & SC + The basal meninges are generally most severely affected, Clinical Features Non-specific prodromal symptoms develop over 2-8 weeks. Stage I (early) [Non-specific symptoms} «© Fever © Lethargy * Headache (may progress to insomnia) Stage 2 (intermediate) [Meningeal stage + do LP] © Confusion - Vomiting * Cranial nerve paresis (CN Ill, IV because lesion is typically at base of brain) + Meningism, vasculitis (hemiparesis, quadriparesis, ataxia, dysarthria) Stage 3 (advanced) [Untreatable stage] * Coma 87Neurology Prepared by Group 433 * Common blood count (anemia, ieukocytosis) © CSE: © Pressure: N/+ © Transparent © Protein: N/t (>1g/L) © Glucose: LOW ; Lymphocyte ' (50-4000/mm*) © Tuberculin test (+-ve in 50% of cases) * CT scan, MRI (hydrocephalus, basal meningeal thickening, infarcts, edema, tubereulom: Prophylaxis: BCG vaccine Treatment - If suspect, commerce antituberculous treatment - Normal regime * Isoniazid (300mg/day) * Rifampicin (600my/day - 2 months) ~ Isoniazid — 6 months + Pyrozinamide (15-30mg/kg/day — 2 months) > Rifampicin ~» 6 months * Drug resistance suspected due to previous antituberculous therapy, add a 4" drug: > Streptomycin (1g/day) or Ethambutal(25mg/kg/day) - Symptomatic treatmentNeurology Prepared by Group 433 (2007) 42) Viral infection, Etiology, pathogenesis, clinical features, treatment, prophylaxis. Invasion of the nervous system may occur as part of a generalized viral infection. Virus enters the body through 1) Respiratory tract 2) GIT 3) Genitourinary tract !) Inoculation through the skin Patient's Ig A neutralizes the —— virus Previous exposure Viral entry No previous. —————> Viraemia exposure Massive viremia ‘Overcome monocyte and Invades CNS via reticuloendothelial defence ~[——> capillaries and veins systems Infection along peripheral nerves «> Invades CNS. After CNS penetration, the clinical picture depends upon the particular virus and the cells of the nervous system which show a specific susceptibility. a) Meninges ~ meningitis b) Parenchyma ~ encephalitis, cerebelitis, myelitis ©) Motor neurons of cranial and spinal nerves ~ poliomyelitis 4) Dorsal root ganglia - radiculitis Some viruses cause a chronic, progressive infection; others remain dormant for many years within the nervous system before becoming symptomatic. Meningi - Isa commonest type of viral infection of the CNS. ~ The term aseptic meningitis includes viral meningitis as well as other form of meningitis where routine culture reveals no other organism. = Develops in late summer. ~ Often affects children and adult < 30, 89Neurology Prepared by Group 433 (21 Common causal viruses - Enteroviruses - Mumps virus ~ Herpes simplex (subtype 2) - Epstein-Barr virus Rare causal viruses - Lymphocytic Choriomeningitis - Human Deficiency Virus (HIV) Clinical features Prodromal phase Meningeal p Recovery + Fever + Headache + 7-14 days + Malaise + Photophobia ° +__Sore throat L:_Drowsiness + Meningeal syndrome a * Hedache Febrile seizure * Vomiting . Ingptensic apeinentier © Nausea * Phonophobia * Photophobia * Meningeal signs (neck stiffness, Kernig = No focal signs = Skin rashes - Parotitis - Diarrhea - Myalgia sign, Brudzinsky’s sign) Enteroviruses ~ Affects children/young adults & occurs seasonally in late sum (Coxsackie/ echo ~ Spread by fecal/oral route viruses) Mumps virus - Affect children/young adults = Winter/spring incidence Accounts for 5% of viral meningitis Y velops in 25% of patients with primary genital infection (su: sexually active adults) ~ Can cause a recurrent meningitis (Mollaret’s meningitis) _ Herpes (subtype 2) plex Investigations he CSF cell count is elevated (lymphocytes or monocytes) with a normal glucose and p: ~ PCR detection of viral DNA/RNA in CSF through diagnostic is rarely thought necessary ~ Virus may be cultured from throat swabs or stool. ~ Serological test on serum in acute and convalescent phases are especially valuable in de: mumps and herpes simplex. Prognosis is excellent and treatment is symptomatic,Neurology Prepared by Group 433 (2007) Encephalitis is inflammatory disease of the brain caused by bacterial or viral infections. - Usually are associated with the visal infection. = Viral encephalitis is a worldwide disorder with the highest incidence in the tropics Encephalitis is subdivided in primary and secondary. -athogenic organism may act: Directly —> primary acute encephalitis. Viral infection causes neuronal and glial damage with associated inflammation and edema, Indirectly via the immune system — secondary, allergic or postinfectious encephalitis and stvaccinial encephalitis. scephalitis following childhood inf and not due to direct viral invasion. jons - measles, varicella, rubella - is postinfeerious ‘ommon causal viruses - Mumps a - Herpes simplex ( Teppre! y - Varicella zoster 4 ee - Epstein-Barr - Arboviruses Clinical features 1.. Signs and symptoms of brain parenchymal involvement - focal. 2. General: pyrexia, myalgia, etc. 3, Specific to causative virus, eg. Features of infectious mononucleosis (Epstein-Barr). ihe focal symptoms depend on the site of the lesion ~ Hemispheres | Coma, confusion, dysphasia, hemiparesis, involuntary movements and epilepsy 2 7 nie ‘Midbrain | Oculomotor palsy, autonomic disturbance Cerebellum | Dysarthria, ataxia Brain stem _ | Cranial nerve palsies, nystagmus, tetraparesis. Spinal cord | Autonomic, motor, sensory dysfunction Etiological treatment is absent, only symptomatic treatment can be provided (except herpetic encephalitis). ( Herpetic infection of the nervous system ‘There are 3 main clinical types of herpetic infections of the nervous system: © Herpetic encephalitis © Shingles (ganglioradiculitis) © Cranial nerves ganglioneuvritis 91Neurology Prepared by Group 433 ( Herpes Simplex types I and 2 and Varicella-Zoster vitus commonly cause dise * HSV-1 - oral and labial rashes as well as encephalitis. * HSV-2 - genital and neonatal infection as well as meningitis. @¢ in humans, Herpetic encephalitis A world-wide disorder occurring during all seasons and affecting all ages. Clinical features General symptoms at onset - Headache > Fever with evolution over several hours to days - Seizures = Impaired conscious level - Psychiatric disorders (euphoria, apatia, disorientation) Focal symptoms: Inferior frontal and temporal lobes are selectively involved: ~ Olfactory or gustatory hallucinations - Behavioural disturbance - . Complex partial seizures - Dysphasia (dominant hemisphere) - Hemiparesis Cerebral edema may result in tentorial hemiation, Investigations Methods of neurovisualisation (help to differentiate with brain abscesses and tumours) 1. CT scan shows low attenuation in the inferior frontal and temporal lobes but may be nor in early stages. 2. MRTis abnormal at an earlier stage, showing bilateral temporal lobe and limbic involveme: often with haemorrhagic changes. 3. CSF examination reveals 6-500 lymphocytes. The protein is mildly elevated and the glucos is normal. IgG is elevated and oligoclonal bands are present. 4. Viral specific antibodies appear in serum and CSF. Treatment Acyclovir - 30 mg/kg/day is given in divided dosage 31/d (to avoid renal toxicity) for 10-14 days, - This treatment has reduced mortality from 70% to 20% with a similar reduction in neurologi sequelae (memory disturbance, ete.) Shingles The virus involves the dorsal root (sensory) ganglion of the spinal cord or cranial nerves ganglion. J ~ Patients are usually > 50 years of age, although it occasionally occurs in ff younger or immunodeficient individuals. Sexes are affected equally. WY - Often occurs in association with compromised immunity, e.g. lymphoma, cancer, AIDS.Neurology Prepai Clinical features ~ Initial signs: * The 1* sign is usually a tingling feeling, itchiness, or stabbing pain on the skin, by Group 433 (2007) * After a few days, a vesicular skin rash appears associated with a burning, painful sensation, Vesicles contain clear fluid and conform to a dermatome distribution. ~ Afler 1-3 weeks, the vesicles crust over and leave irregular skin depigmentation with scarring, ranial nerve gangl ec a Trigeminal | Usually ophthalmic division with vesicles above the ulceration - herpes zoster ophthalmicus eye and associated corneal | Geniculate | Vesicles within the external auditory meatus and e Diagnosis: Based on clinical features. Prognosis ‘Isually resolves without complications. The rash and painful usually go away within 3 to 5 eek, act with a person with shingles may cause chickenpox (but not shingles) in someone who has never had chickenpox before Treatment ~ Depends on the severity and location of skin lesions, Mild disease requires symptomatic treatment only. ~ Severe diseases, involvement in immunodeficient patients or ophthalmic. vesicles require acyclovir either orally or intravenously. Poliomyelitis ~ An acute viral infection in which the anterior horn cells of the spinal cord and motor nuclei of the brain stem are selectively involved. - A major cause of paralysis and death 30 years ago, now rare with the introduction of effective vaccines. Causative viruses ~The poliovirus is an enterovirus. - Coxsackie and echoviruses may produce a clinically identical disorder. Mode of spread Spread by faecal/ oral route Once ingested the virus multiplies in the nasopharynx and GIT. Incubation ranges from 5 to 35 days (average 7 to 14 days). PPE proportion (2%). Penetration of GIT results in viraemia but CNS involvement occurs in only a very small 93Neurology Prepared by Group 433 (2007 Clinical features There are 4 stages of the polio. Afier ez 1) Subclinical stage + resul | 2) Mild non-specific s + Fever, occurring 5 to 7 days + Sweating * Malaise + Headache * Mild gastrointestinal upset 3) Serous meningitis (may be as preparalytic stage) | + Severe headache + Features of meningism + Back and fimb pain + Muscle tenderness Brain stem form (motor clei of cranial nerve are involved) ~> Pharyngeal, laryngeal, lingual and facial weakness, + Spinal form (anterior horns are involved) + Paralysis develops: widespread or localised; ascending or descending, often proximal and asymmetrical, maximal development during 24 hours + __May be mono-, para- or tetraparalysis, 108i 1. The clinical picture + 2. CSF examination (may be normal or polymorphs and lymphocytes increased; protein 7 with normal glucose) are sufficient to reach the diagnosis. 3. Serological tests + viral cultures of throat washings, stools, | F will confirm later. Prownosis - Inepidemies, a mortality of 25% results from respiratory paralysis ~ Improvement in muscle power usually commences one week after the onset of paralysis ane continues for up to a year. - Only a proportion of muscles remain permanently paralyzed. - _Inaffected limbs, bone growth becomes retarded with shortening as well as thinning. ‘Treatment - Bed rest & fluid balance carefully maintained. - Respiratory failure may require ventilation. ~ Avoid the development of deformities in affected limbs with physiotherapy and splinting ~ Aim of treatment: To control symptoms while the infection runs its course.Neurology Prepared by Group 433 (2007) Post-Polio Syndrome A significant proportion of polio patients develop late sequelae oflen 30 yrs after initial illness « fatigue, myalgia and new weakness are characteristic. Prophylaxis Sabin vaccine (vaccine of choice) — live attenuated virus given orally :man immunodeficiency virus (HIV) ‘ological disease affects 1/2 to 2/3 of patients and can occur at any stage of the infection. In some cases they may be initial manifestation of AIDS (acquired immunodeficiency syndrome) Classification —_ ee Primary HIV infection of the nervous system Acute reversible encephalopathy Acute aseptic meningitis Cranial neuropathies Acute myelitis AIDS dementia Toxoplasma gondii Cryptococcal meningitis seit - Herpetic infection _ ‘oplasm * Pri CNS lympho: Kaposi sarcoma ___ Opportunistic infections of CNS Luberculous meningitis: ~ Always secondary. The primary focus of infection is usually in the lungs. ~ Morphological changes are usually most intense at the base of the brain; Clinical symptoms ~ Subacute onset (days or weeks): progressing headache, vomiting, fever, disorder of sleeping (lethargy) - Meningeal syndrome ~ Cranial nerve palsies (due to basal inflammatory process) - CFS: lymphocytic pleocytosis + low glucose 2 &Neurology Prepared by Group 433 (2 43) Varicella~ infections. Pathogenesis, clinient fentures, treatment, Varicella (chickenpox) and herpes. zoster (shingles) are different clinical manifestation infection by the same virus ~ Varicella-Zoster, a DNA human herpes virus. Etiology ~ an acute encephalitis - viral meningitis - myelitis ~ postinfectious encephalomyelitis - postinfectious polyneuropathy (Guillain-Barre syndrome) Shingles occurs after virus reactivation, dormant afler the primary infection (chickenpox). Pathology - The virus involves the dorsal root (sensory) ganglion of the spinal cord of the cranial ac sensory ganglion- trigeminal or geniculate. - The inflammatory process may spread into the spinal cord and involve posterior and anter horns. - Similarly inflammatory changes may occur in the brain stem. Clinical features = Patients are usually >50 years old. 9=d. - Recurrence is rare, - Often occurs in immunocompromised patients eg. Lymphoma. Also associated w spinal/nerve root trauma, — Initial features - Ayesicular skin ash associated with a buming, painful sensation: - Vesicles contain clear fluid and conform to a dermatome distribution. - After 1-3 weeks, the vesicles crust over and leave irregular skin depigmentation with scarti - Motor weakness occurs in 20% due to damage of the anterior horn cell. - More widespread spinal (myclitis) or encephalic involvement occurs in the immunodeficie: - Inthese patients extensive cutaneous lesions are common (disseminated zoster). Cranial nerve ganglia involvement ae Trigeminal | Usually ophthalmic division with vesicles above the eye ulceration > HERPES ZOSTER OPHTHALMICUS . Vesicles within the external auditory meatus and eardrum. Ipsilateral deafness weakness resul Y HUNT SYNDROME. ‘and associated co Diagnosis ~ Based on clinical features. - Virus DNA can be detected in vesicular fluid by PCR. ‘Treatment = Depends on the severity and location of skin lesions. - Mild disease requires symptomatic treatment only. ~ Severe disease, involvement in immunocompromised patients, or ophthalmic vesicles rea acyclovir either orally/lV.Neurology Prepared by Group 433 (2007) 44) Neuro AIDS. Etiology, pathogene: differential diagnosis. cal features, treatment, prophylaxis and Definition of Neuro AIDS Affection of nervous system due to AIDS > cause complications. Priology @) Primary ~ Direct attack of NS by HIV 'b) Secondary ~ From opportunistic infections, tumors, drug related complications rogenesis LILV neuroinvasi HIV enters brain via infected monocytes and CD4+* cells and infects other surrounding cells like microglia, astrocytes, oligodendrocytes and neurons b) HIV proteins (virotoxins) Damage to neurons by actions of specific HIV proteins (gp120, gp41, Tat, Nef, Vpr, Rev). May be directly toxic to neuro cells or cause damage by activating astrocytes, microglia and macrophage to produce cytokines, chemokines, immune disease CNS damage by humoral immune mechanisms eg. Presence of anti-CNS antibodies in AIDS patients with dementia. Clinical features 1). Primary affection of NS i. AIDS dementia Encephalitis; behavioral changes;- gradual: decline in cognitive functions ~ memory, concentration; progressive slowing of motor function and loss of dexterity and coordination. ii, Peripheral neuropathy Numbing, burning or tingling sensation that begins in legs and feet; heighten sensitivity to pain, touch or other stimuli; present muscles wasting, muscles weakness, fasciculation, reduced or absent tendon reflexes. iii. Vacuolar myelopathy Protective myelin sheath pulls away from nerve cells of spinal cord, forming vacuoles in nerve fibers. Symptoms: Weak and stiff legs, unsteadiness when walking. 2) Secondary affection of NS i. Opportunistic infections of CNS a) Toxoplasma gondii ~ cause encephalitis b) Cryptogenic meningitis c) Herpes zoster ~ causes shingles d) Neurosyphillis ii, Primary CNS lymphoma ~ Associated with Epstein Barr virus (EBV) o7Neurology Prepared by Gre ap 433 (200° ‘Treatment + Highly active retroviral therapy (HAART) — combination of: 2 nucleoside analogs + 1 from non-nucleoside transcriptase inhibitor or protease inhibitors © Antiretroviral with good CSF etration Lamivudin, stavudin, zidovadin, efavirenz, nevirapin, indinavir - Pain relief: Analgesics, anti-epileptics ~ Anti- inflammatory: Corticosteroids. - Anti- depressants: Amitriptylin - Anti- dementia - Antibiotics Prophylaxis: Same as AIDS Differential diagnosis ~ Alzheimer disease - Parkinson disease = Multiple sclerosis - Metabolic encephalopathies - Vitamin B12 associated neurological diseasesNeurology Prepared by Group 433 (2007) 45) Transient ischemia attack (TIA). Aetiology, pathogenesis, clinical features, treatmen, prophylaxis and differential diagnosis. TIA ~ Episodes of focal neurological symptoms duc to inadequate blood supply to the brain ~ Attacks are sudden in onset, resolve within 24 hours or less. ~ Leave no residue deficit (reversible). = Important warning signs of cerebral infi Etiology Embolism Fhrombolism Hypertension Stenosis of the vessels \therosclerosis pM 2 Heart disease ie, Atrial fibrillation Pathogenesis ~ A fof cerebral blood flow <20-30mL/100g/min produces neurological symptoms. It is mainly due to the fall in perfusion pressure (haemodynamic) and blockage of the passage of flow by embolism(atise from plaques). ~ The degree of infraction is based on the degree of reduction of flow and the duration of such a reduction, If flow is restored to an area of brain within the critical period, ischemic symptoms will reverse themselves. Clinical features ~ All the symptoms have sudden onset and last-a short-time ie. 2-30min-or more (seldon:>1-2hs), then abate without persistant neurologic abnormalities, consciousness remaining, intact throughout episode. + Clinical picture depends on vessels involved, Anterior carotid territory Contralateral hemiparesis, contralateral hemisensory disturbance, dysphasia, monocular blindness (amaurosis fugax). Posterior vertebrobasilar territory Loss of consciousness, bilateral limb motor/sensory dysfunction, binocular blindness, (vertigo, tinnitus, diplopia, dysarthria — in combination) ‘Treatment + Exercise + [risk factors ~ Antiplatelet (Aspirin 30mg/d) + Anticoagulants (Heparin) - Nootropic ~ Antioxidants ~ Surgical treatment, if the stenosis is >75% PS: Itis obligatory to hospitalize to determine more concrete outcome, ferential diagnosis ~ Strokes 99Neurology Prey 46) Intracerebral Hemor 4 ology, Pathogenesis, Clinical Features, ‘Tre: Prophytaais and Differential Diagnosis. : ed by Group 433 Classification of intracranial hemorrhage: 1) Epidural hemorrhage 2) Subdural hemorthage 3) Subarachnoid hemorrhage 4) Intracerebral hemorrhage Intracerebral Hemorrhage Pathologic extravasation and accumulation of blood within the brain substance with pr Tupture through the cortical surface (pseudosubarachnoid hemorrhage) or into the ventric system (intraventricular hemorrhage) producing stroke syndrome. ——— Stroke syndrome ~ Acute onset offheurologic deficil that persist for at least 24 hours, eto oy CNS that is result oF disturbances of cerebral ci ~ 15% of all stroke cases. involvement @ Etiology ~ Hypertension ~ Cerebral vascular disorders eg. cerebral amyloid angiopathy, cerebral artery atherosclerost ruptured aneurysm, arteriovenous malformation, vasculitis ____—~ Neoplasm Trauma eg, cerebral contusion, concussion - Hemorthagic diathesis'e.g. hemoph ~ Anticoagulant induced ~ Drug abuse eg. cocaine ~ Idiopathic ieee (Pathogenesis Direct trauma~or cerebral arterial diseases|the integrity of endothelium -~ alternating 5! (hypertension) causes blood to dissect through the weak point, altered blood propert (anticoagulants) inhibits normal coagulation and hemostasis -- rupture of vessel walls extravasation of blood into brain substance -> accumulation of blood in the brain substance accumulation of blood in the brain substance Effect of blood accumulation DY) Space occupying effect - Hematoma formation causes displacement of brain substance causing herniation ic subfalcine, transtentorial, tonsillar (maybe fatal because causes compression of medull oblongata). ) - Compression by hematoma causes (direct dysfunction pf the surrounding structure and their vascular component, leading to deat dar rae) and edema, - Hematoma may continue io expend over the 1* few hour due to continuous bledding.Neurology Prepared by Group 433 (2007) 2) Neurotoxicity of blovud Blood and plasma acts on surrounding brain substance causing distuption of blood-brain barrier, vasogenic and cytotoxic edema that causes neuronal pannectosis and edema 3) Canity formation - Occurs after resolution of hematoma (after 4 to 8 weeks) - Permanent loss of neurons and neurological functions. Clinical Features = Depends on the side of hemorrhage and formation of hematoma © Supratentorial hematoma * Cerebellar hematoma * Pontine hematoma - In hypertensive pts, hemorrhage usually in basal ganglia/thalamic region Symptoms and Signs 2 Hesdache Pe edema ca Positive meningeal syndrome = Rapid loss or gradual deterioration (over 48 hours) of consciousness - Hemiparesis: ~ - Hemianesthesia~ ~ Homonymous hemianopia. - Oculomotor nerve palsy symdrome ~ indicates transtentorial herniation - Limb weakness prior to loss of consciousness Cerebellar hematoma ~ Sudden onset of headache : - Cerebellar syndrome eg. cerebella ataxia, cerebellar nystagmus, vertigo, dysarthria, dysdiadochokinesia, muscular hypotonia, tremors and dysmetria, asynergic Babinsky’s sign, “rebound” phenomenon etc. - Brain stem syndrome eg. depress level of consciousness, bulbar syndrome, nausea, vomiting, depression of respiration e.t.¢. ~ Signs of TICP and hydrocephalus caused by CSF obstruction. Pontine hematoma - Sudden loss of consciousness potine * 7 - Quadraplegia unes - Hespiatioe irregularities (usually depressed respiration) s a n lass conseso - Fever— Qquadrapleglé + Miosis — ye ver ~ Dysconjugate ocular movements ~ ih + Bulbar syndrome ee ~ Usually fatal, —~ 101Neurology Prepared by Group 433 (2007) Laboratory and Instrumental investiga 1) Lumbar puncture - Review massive erythrocytes (if the hemorshage extended to the ventticles) - Presence of other blood components eg. PMN cells ete. 2) CTMRI set = Determines the exact size and location of the hematoma - High plane may review hydrocephalus ~ Review displacement of ventricular homs (usually increase distance in thalamic region hemorrhage) 3) Cerebral angiography + Inurgent pre-operative state for identification of secondary causes eg, AVM, rupture aneurysin or vasculitis = Hf not usually dove after resolution of hematoma for visualization of post resolution vascular flow - Ifangiography reviews negative result * late MRT may review carvano Management Regimen: Strict bed regimen Dict : Usual unless indicated Medical Symptomatic treatment * Oxygen through mass, intubation maybe needed in case of depressed respiration # Analgesics * Support of blood pressure (monitor CVP) and appropriate interventions * Antiepileptics (PRN) * Neuroleptics © Nootropics ¢ Adaptogens angioma, Etiologic © Blood pressure control: ACE, iz-adrenergic antagonist * Proper blood glucose control. == ® Lipid control: Statins, fibrates Surgical * For supratentorial hematoma — with craniotomy flap approach © Superficial hematoma Deterioration because of compression effect © Size of hematoma not <3mm in diameter ‘* For cerebellar hematoma ~ with suboccipital craniotomy approach © Large hematoma © Eminent signs of brain stem compression. * Mortality of pontine hematoma is high. Conservative approach is usually adopted. * For intraventricular hemorrhage ~ with ventricular drainage or shunt © Presence of obstructive hydrocephalus © Conservative management — ThrombolyticsNeurology Prepared by Group 433 (2007) 47) Subarachnoid hemorrhage. Aetiology, pathogenesis, clinical features, treatment. Subarachonoid hemorrhage (SAIL Bleeding from intracranial vessels in subarachnoid space. Etiology ~ Aneurysm: Artherosclerosis, congenital anomalies Atferiavenous malformation Hypertension Biveding diathesis - Anticoagulants - Vasculitis - Tumor - Undefined Rupture of vessels in subarachnoid space Clinical features - Headache: » Acute onset, diffuse and severe, like ‘blow’& ‘explosion’ in head © Duration: Many days + May spread to neck, back as blood track down into spinal subarachnoid space - Transient or prolonged loss of consciousness or epileptic seizure ~ Vomiting and nausea ~ Meningeal syndrome: * Develop after 3-12 hours * Act as meningeal irritant - « Neck stiffness, Kernig’s sign, Brufinski’s sign - Reactive hypertension ie. Rise in BP without evidence of pre-existing hypertension, and take several days to return to normal level. - Papilloedema - Absent focal neurological symptom Laboratory diagnosis - CT scan: If negative, then performed lumbar puncture. - Lumbar puncture (LP) © Should be done only after 6 hrs since the onset. * Required to identify characteristic yellowish colour (xanthochromic) of centrifuged CSF due to breakdown of hemoglobin. * Normal LP excludes diagnosis of SAH. = Cerebral angiography = MAbs WAY? + 103Neurology peresee/& Prepared by Group 433 (20079 ‘Treatment = Bed regimen: 40 days ~ Hemodilution: 2-31 of fluid per day ~ Anticonvulsion therapy: Phenyton 300-400mg/d (Maintain plasma level at 10-20inpéml.) ~ Homostatic treatment, eg. Vikasol, aminocaptine Analgesic (ie. Headache) Control BP, eg. antihypertensive drugs Antiepileptic drugs which depend on situation. Prevention secondary ischemia; Nimodipine 60mg PO, every 4 lr for 21 days ~ Surgical: * For patient with aneurysm who is alert, oriented, no focal deficit ® Performed at 10-14 days * Surgical clipping, coiling, ligating: to | re-bleeding and improve survival 104Neurology 48) Ischemic stroke. Types (embolism and 1 Prepared by Group 433 (2007) rombosis). Etiology, pathogenesis, cl features, treatment and differential diagnosis. ischemic stroke mignon — Accounts 80% of stroke ~ Subdivided into a) Large artery atherothrombosis ©) Lacunar stroke b) Brain embolism d) Systemic hypoperfusion Arterial dis ial fibrillation ¢ (stenosis) | Pathogenesis + Valvular heart disease * Diabetes Mellitus + LV thombosis © Smoking + Cardiomyopathy # Hyperlipidemia/atherosclerosis * Coronary artery disease * DiC/coagulopathy «Aortic archplaque b“Hypertension ‘© First 3-6 hr Penumbra_period: no morphological changes but {x absent, mostly | ‘caused by occlusion of cerebral artery Panckion L chawg ¢ After 6 hr -> Focal infarction 3c V og\e6 >48 hr — Visible focus on CT scan Ischemic penumbra is an area of brain that reached the reversible stage of neuronal failure. Tissue is potentially salvageable & should be resou carly reperfusion. Clinical = ‘© Onset of neurological deficit preceded |» > 60 y.o. picture by TIAs in different artery. © Onset of neurologic deficit preceded by © Sudden in 80%, with max deficit. at). TIAs in 50%: onset, other show stepwise:| «Often occur during sleep (awakens with progression. neurological deficit). © Middle cerebellar artery syndrome-is | Neurologic changes often fluctuate in a the most common presentation | stepwise progressive or remitting (contralateral, sensorimotor deficit, | fashion due to recanalization, aphasia ~dominant hemisphere). rethrombosis & changes in collateral _ _ |__blood flow. _ ‘© Depends on the vessel involved: © Amaurosis fugax or permanent iz (Hemisscalhes fen tty poosthcila psuparkadial sensation Loss © Hemiparesis 3 ema deer ret © Dysphasia 7 Remonenes benagig beMoe © Focal or generalized seizures contra beAraL ile ©__| consciousness _ TS Treatment © Warfarin ‘* Thrombolytic therapy from onset < 3hr © Aspirin © Aspirin + heparin for others © Antiarrhythmic treatment * Carotid endacterectomy (or stending) © Aneurysmectomy for moderate or severe stenosis ‘© Repair of atrial septal defect * Resection of atrial__~— myxoma embolization _ Differential “|e CT scan to exclude intracerebral hemorrhage and tumour diagnosis © ‘Ischemic salvage’ on MRI 105Neurology Prepared by Group 433 (2007 49) Multiple sclerosis. Etiology, pathogenesis, it features, (reatment. Multiple Sclerosis ~ Is a demyelinating disease normally characterized by focal disturbance of fumetion relapsing and remitting course. ~ Distribution: 9>3, common in temperate climates Etiology: Unknown, multifactorial disease. Pathology & Pathogenesis Presented as scattered lesion with greyish colour from 1mm to cm in white matter of brain or Sc (plaques). The lesions lic in close relationship to veins (postcapilllary venules) - perivenow distribution, RECENT LESIONS > LATER > OLDLESIONS Myelin destruction Astrocyte proliferation Relatively acellular and Relative axon sparing more clearly dematcated Perivenous infiltration with Within these plaques bare mononuclear cells and . @xons are surrounded by lymphooytes, Interstitial astrocytes. ‘edema is evident in acute Axon loss accounts for lesions, Increasing disability. Breakdown of blood-brain barrier occurs and may be essential for myelin destruction, , Immune deficiency ~» possible persistence of a latent virus and variations in immune status could be the bases of relapses and remissions. T-lymphocytes and macrophages found in plaques may be sensitized to myelin antigens. In summary ~ the causation is probably multifactorial Lagi exposure: * Genetic predisposition “s Disordered autoimmune response * Environmental exposure (viruses) © Age of individual at exposure a bow a viola Clinical Features MS is usually characterized by: * Signs and symptoms of wide spread white matter disease * A relapsing and remitting or progressive course 106Neurology Prepared by Group 433 (2007) ‘Symptoms at onset 1) Vague symptoms: * Lack of energy Headache Depression Aches in limbs May result in psychoneuroses 2) Precise symptoms: * Sensory disturbance ~ 40% * Retrobulbar neuritis — 17% + Limb weakness ~ 12% © Diplopia- 11% Vertigo ~ 20% Ataxia —20% Sphineter disturbance - 20% ‘trigeminal neuralgia may be an early symptom of MS, should be considered in young patient with paroxysmal facial pain. Sensory symptoms * Numbness and paresthesia —> ofien due to posterior column demyelination than to spinothalamic tract involvement « Impaired vibration’ and join. position sensation.» posterior column. Jesion (no position awareness) » Lhermitte’s sign — cervical posterior column involvement —* sudden neck flexion will evoke shock like sensation in limbs * Dysaesthesia — spinothalamic lesion © Loss of all sensory modalities -> posterior root entry zone lesion Motor Symptoms * Mono/paraparesis -most common * Paraparesis > result of spinal demyelination at cervical region Signs: }tone, hyperactive tendon reflex, absent abdominal reflex, extensor plantar response Loss of vision Acute optic neuritis (Retrobulbar neuritis): © Visual loss with central scotoma associated with pain on ocular movement and recovery over some weeks. Milder form: only colour vision affected. * On examination: Papillitis Disturbance of ocular movements © Diplopia (affects CN LI, IV, VI) © Nystagmus * Pupil dilation despite presence of direct light 107Neurology Prepared by Group 433 * Vertigo of central type Alaxia of gait (cerebellar or sensory type) Intentional tremor, dysarthria ~~» cerebellar involvement Mood change: Euphoria, depression Emotional lability: Crying/ laughing Sphincter disturt Paroxysmal: Paraesthesia, dysarthria, ataxia, eee ere t, epilepsy, photopsia, trigeminal neuralgia Treatment: SYMPTOMATIC © Spasticity: Drugs that act at spinal or skeletal muscle level Baclofen - GABA derivative Dantrolene - direct acton on muscle Tizanidine ~ a adrenergic agonist Urinary symptoms: Anticholinergies, desmopressin s| Bowel symptoms: Lactulose, loperamide Pain: Analgesics, anticonvulsants, antidepressants, NSAIDs Paroxysmal symptoms: eg. tonic seizures ~ anticonvulsants Fatigue/mood change: Amantadine, SSRis Tremor/ataxi: lockers: ey 108Neurol Prepared by Group 433 (2007) 50) Acute demyelina treatment, cacephatomyelitis. Etiology, pathogenesis, clinical features, 1s a neurological disorder, which caus 's a short and sudden inflammatory attack of the brain and spinal cord that damages the myelin sheath ~ the fatty tissue that protects nerve cells. ~ Is an acute widespread demyelinating condition, which principally afte cord. ~ Usually follows an infection or vaccination, ~ Is characterised by multifocal white matter les fons on neuroimaging. ~ Isa monophasic disease. ~ Uncommonly ADEM caa relapse frequently. If these relapses are thought to represent part of the same acute monophasic illness, the term multiphase ADEM is used ts brain and spinal Etiology ~ In developed countries most commonly follows non-specific Upper respiratory tract infection, common childhood illnesses (eg measles, chickenpox) “st immunization encephalomyelitis, * Most commonly associated with measles, mumps and rubella vaccination * Incidence of ADEM following measles vaccination is 20 times lower than the incidence after measles infection. Pathogenesi ADEM is thought to be an autoimmune disorder of the CNS. - The mechanism» proposed. is that myelin autoantigens, such as myelin. basic protein, Proteolipid protein and myelin oligodendrocyte protein, share antigenic: determinants with those of an infecting pathogen. - Antiviral antibodies or a cell mediated response to the pathogen cross react with the myelin autoantigens, resulting in ADEM. * Pathology ~ No change occurs in the external appearance of the brain or spinal cord, ~ Loss of myelin, with relative sparing of the axon, > Microscopie: Perivenular lymphocytic and mononuclear celt infiltration and demyelination "Other changes include Ayperaemia, endothelial swellaig, and vessel cis invasion. by inflammatory cells, perivascular vedema, and haemorrhage + These changes are present in the small Blood vessels of both white and grey matter. ~ As the lesions become older, the macrophages increase and lymphocytes decrease in number. ~ Ala late stage of disease foci of fibrillary fibrosis can also be seon ia adjacent brain tissue . 109Neurology Prepared by Group 433 (2007 Clinical features ~ Symptoms usually begin 1-3 weeks affer infection or vaccination Major symptoms are fere headache, drowsiness, seizures and coma, ~ Non-specific symptoms (eg. fever, malaise, myalgia, headache, nausea ancl vomiting) of Precede neurological features, beginning 4-21 days afier precipitating event - Neurological features ~ Focal or multi-focat ~ Rapid onset over few days ~ Early features: + Encephalopathy: ranges from lethargy to coma + Hemiparesis * Cranial nerve palsies * Paraparesis. Cord lesion often complete ~ Meningism ~ Ataxia - Movement disorders ~ Fits in severe cases — Optic neuritis ‘reatment - IV methyprednisolone or ACTH associated with clinical improvement. * Shorten duration of symptoms and immediately halt further progression * 2/3 of patients show a response, ~ Plasmapheresis’ and IV immunoglobulin reported: tbe. associated with dramatic improvement in some patient who does not tespond to corticosteroids ~ Cytotoxies 110Neurology Prepared by Group 433 (2007) 51) Parkinson’s disease. Etiology, pathogenesis, clinical features and treatment. Etiology ~ Described by James Parkinson (1817) = Cause is unknown - Annual incidence (20 per 100000); d:9= 3:2 ~ Ave of onset” 50 yrs upwards, often 60 y.o. ne mutation in young onset & familial cases (synuclein & parkin) vironmental factor (eg. Smoking/herbicide exposure) ‘nies: typical parkinsonian syndrome “athogenesis - |dopamine [ Dopamine -> inhibitory effect] - Hypertonicity-hypokinetic syndrome = Substantia nigra contains pigmented cells (neuromelanin) -> black appearance; These cells lost in Parkinson’s disease; substantia nigra becomes pale. - Remaining cells contain atypical eosinophilic inclusions in cytoplasm (Lewy bodies); they are found in cerebral cortex esp. when dementia is present (diffuse Lewy body disease). Clinical features Pill lig! ‘tremor of hands; occurs at rest, improves with movement and disappears during Ri iidity -> Flexed posture radykinesia: Slowness of voluntary movement © Mask-like appearance (face expression); hypomimic Speech ie monotone Dysarthria Dysphagia Slow deliberate gait with little associated movement (eg. Arn swinging) Tremor, rigidity, bradykinesia — deteriorate simultaneously, affecting every aspect of the patient’s life: © Micrographia © Stiff, shuffling gait © Seborthea (greasy skin) © Rising from a chair becomes laborious with progressive difficulty in initiating lower limb movement from a stationary position Eye movement affected (loss ocular convergence & upward gaze) © Depression, dementia (in 30% patient) © Hypertonus ie Cog-wheel . MWNeurology Prepared by Group 433 (2 ‘Treatment - Symptomatic & » halt the pathological process, - Itaims at restoring the Dopamine/Acetylcholine balance {dopamine deficiency). dopamine 1) Exogenous dopa (levodopa or levodopa + decarboxylase) 2) Dopamine agonist (bromocriptine, lisuride, pergolide) ~ Exogenous dopa improves bradykinesia, rigidity & to a lesser extent, tremor, but in 20%! response is poor) - Central S.E.: confusion, depression, dyskinetic movements - When levodopa responsiveness is lost, dopamine agonists are used. Basic treatment 1) Exogenic dopamine ~ levodopa 2) Anticholinergic drugs 3) Dopamine agonists 4) MAO-B-inhibitors: Breakdown of dopamine -> {dopamine levels 5) Amantidine (antiviral drug): help in rigidity 6) Stereotactic surgery ~ Lesion of the globus pallidus or thalamus)Neurology Prepared by Group 433 (2007) 52) Huntington's disease. Etiology, pathogenesis, clinical features, treatment. Definition A degenerative brain disorder which gives rise to progressive. selective (localized) neural death which associated with choreic movements and dementia. Etiology Genetic disorder ~ Autosomal dominant trait, associated with increase in length of CAG triplet repeat ina gene called ‘huntingtin’ located on chromosome 4p16.3 Pathogenesis Neuronal loss in striatum associated with reduction in projection to other basal ganglia siructure (Eg. Atrophy of caudate nucleus which form lateral margin of lateral ventricle > arise in middle and late stage of disease). Lost of cells in deep layers of frontal and parietal cortex (corticostriatal projection). - Deficiency of GABA and acethylcholine with reduced in activity of enzymes glutamic acid decarboxylase (GAD) and choline acetyltransferase (CAT). cl cal features Classic signs: a) Progressive chorea~ Maybe initial symptom that progress from fidgetiness to gross involuntary movements which interrupt voluntary movements —> feeding and walking difficult. 5) Rigidity ) Dementia~ Subcortical type: apathetic, forgetful-and slow, poor ability to use knowledge. (Typically, there is a prodormal phase: of mild psychotic and behavioral: symptoms which preceeds frank chorea by up to 10 years) + Behavioral disturbances— Personality change; affective disorders and psychosis. * Primitive reflexes- Grasp, pout and palmomental. Eye movements disturbed with impersistence of gaze, Hees ) Phenothiazide 2) Haloperido! — Antipsychotic, block Dz-dopamin receptor in limbic system 3) Tetrabenazine 4) Benzodiazepine group (neuroleptics) ~ Diazepam, oxazepam 13Neurology Prepared by Group 433 (2 53) Vascular diseases of spinal cord. Aetiology, pathogenesis, clinical features treatment. ‘The blood supply of the spinal cord Posterior spinal arteries ‘+ From posterior inferior cerebellar arteries * Form pial arteriolar plexus on the posterior surface of the spinal cord ‘© It supplies posterior 1/3 of spinal cord and dorsal column Anterior spinal artery * Branches from each vertebral arteries unite ta form a single vessel iying in the median fissure of the spinal cord * Penetrating branches: anterior and part of posterior grey matter ‘¢ Circumferential branches: anterior white matter © Central (sulcal) arteries: anterior 2/3 of spinal cord Etiology a ~ Atheromas - Hypotension - Inflammatory vascular diseases ~ Collagen vascular diseases ~ Thrombolism - Embolism ~ Blood diseases - Compression of spinal arteries by tumor, aortic aneurysm ete Vascular Syndromes > Anterior spinal artery syndrome = Posterior spinal artery syndrome = Spinal infarction ~ Spinal hemorrhage ~ Dural/perimedullary arteriovenous (AV) fistulaNeurology Prepared by Group 433 (2007) Anterior spin | spinal artery synd + Local or radicular back pain + Pain in the spine + Flaccid limbs — spastic | + Sensory impairment + Areflexia — hyperreflexia Paresthesia in the legs + Sensory impairment Loss of position and vibration sense — Loss of pain and temperature sensation - Loss of deep tendon reflexes + Motor impairment Para/ tetraplegia Spastic weakness of the legs~ unable to walk + Incontinence of urine and feces UMN or LMN weakness Spinal infare = c “Route spi cord transection syndrome with | Ifhemomhage is small flaccid paraplegia or quadriplegia — There may be only spastic weakness associated vial sensory loss below the level of the lesion with hyperreflexia in the legs and bladder hineter dysfunction dysfunction nomic dysfunction may also occur sually it is due to acute occlusion of the great | If hemorrhage is large radicular artery (of Adamkiewicz). = Signs of cord tansection include flaccid paralysis, complete sensory loss below the lesion, absent reflexes, Babinski signs, and loss of sphincter control Dural/perimedullary arteriovenous{AV) fistula + An abnormal: communication: (shunt) between an artery.and vein between the two: layers of the dura mater, + An arterial: branch. of a’ spinal’ artery feeds directly: into -a: superficial: spinal-veiny: which therefore: contains arterial rather than venous:blood, flowing in the opposite direction to normal: + It appears as a mass of convoluted dilated vessels, Clinic + Paroxysmal stabbing pain * Episodes of slowly progressing paraparesis, + Sensory loss separated by periods of remission Diagnosis = MRI, CT, Shadow spine radiography, Myelography Treatment = Symptomatic therapy ~ Receive fresh whole blood and vitamin K = Surgical reconstruction: Embolisation, decompressive laminectomyNeurology Prepared by Group 433 5d) Syringomyeti ology, Pathogenesis, Clini features, Is a chronic progressive condition of the SC, in which a cyst (syrinx) forms within, exp: elongates in the SC, causing formation of longitudinal cavities lined by dens other perforating neurological tissues and their associated neurological deficits liogena Synonyms: Morvan’s disease, hydrosyringomyelia, cystic myelopathy Communicating Syringomyelia Syrinx communicates with the central canal (hydrodynamic disorder of the CSF pathways) “Non-communicating Syringomyelia Cystic dilation of spinal cord without communication with central canal. Etiology _ — ampenganaaes = Communicating syringomyelia — often associated with developmental anomalies of brainstem and foramen magnum region: * Arnold-Chiari malformation type I (adult onset) ¢ Chronic arachnoiditis of basal cisterns Nou-communicating syringomyelia — ofien due to: © Posttraumatic syringomyelia (PTS) ¢ Intramedullary tumors (tumors in the SC)- Astrocytoma, glioblastoma multiforme, PNET ¢ Spinal arachnoiditi: Pathogenesis Arnold-Chiari_ malformation: Cerebellar tonsillar herniation into foramen magnum —~ obstruction — syrinx formation — syringomyelia Posttraumatic syringomyelia: (mullifoctorial): * Liquefaction of intraparenchymal hematoma ¢ Ischemia due to tethering (sequel of bleeding stenosis, or kyphotic deformity) © Arterial or venous obstruction * Mechanical damage from cord compression * Release of intracellular lysosomal enzymes and excitatory amino acids Cavity formation —+ Enlargement and extension of the cystic cavity (rostral or caudal extension occur due to turbulent CSF flow or a "one-way valve" phenomenon that allows int Lonly of CSF into cavi induced arachnoiditis, scarring, spinalNeurology Prepared by Group 433 (2007) Clinical Features + Depends on site of cavitation! ~ Cavitation commonly occurs in cervical region (Armold-Chiari mallormation type 1) General - Dissociated sensory loss coincides with level of lesion. May be reflected by presence of painless skin ulcers, scars, edema, hyperhidrosis, neuropathic joints, resorption of terminal phalanges ete. © Atlevel of lesion ~ LMN lesion syndrome © Below level of lesion - UMN lesion syndrome Typical cervical syringomyelia symptoms * Cape like distribution of sensory loss over shoulder (uni/bilateral) Diffuse pain in neck * Radicular pain in arm ‘Springobulbia — syrinx formation in the lower part of brainstem. — * Ipsilateral tongue atrophy * Vocal cord paralysis~ Dysphonia * Palatal weakness * Dissociated trigeminal sensory loss (anesthesia in skin of nasal region), + Other S/S of brainstem involvement eg: Bulbar syndrome. Typical for Arnold-Chiari malformation © Late onset symptoms: 25 to 40 y.o. ¢ Symptoms are worsen with straining or any activity that causes CSF pressure to fluctuate suddenly * S/S of cerebellar involvement (cerebellar ataxia, nystagmus etc.) © Obstructive hydrocephalus Brainstem compression (bulbar syndrome) or individual lower CN involvement * Arachnoiditis and syringbulbia "Typical for PTS * Typical cause of PTS: Car accident or trauma involving a whip-lash injury * May have long subclinical period * Symptoms usually limited to ipsilateral manifestations, but may also adversely affect sweating, sexual function, and, later, bladder and bowel control _ 7Neurolog Prepared by Group 33 By P iagnostic ~ MRI, CT sean, myelography, electromyography on the cervical segion and the magnum region, ~ Wany indication of trauma, malignancies or inflammatory diseases, full spinal panc! done. ~ Case 10 case basis for lumbar puncture eg chronic arachnoiditis, Treatment Depends on underlying cause, Regimen ~ Hospitalization and bed regime (immobilization PNR) Diet| — Normal (restrict Na’ intake if increase Ic) Schedule for neurological and neurosurgical consults, Schedule for appropriate physiotherapy and tehabilitations ‘Temporary/emergeney telieve: Decompression of distended syrinx by puncture. ‘ommunicating syrin; Non-comm) unicating syringomyelia © Shunt placement for syrinx drainage + Excision of tumor for benign tumor and carly stage malignancies * For PTS: Recommend surgery only in ease at brogressive:neurological deficit or into’ © Various drainage 9° Myelotomy © Surgical meningocele formation Medical treatment * Analgesics or antiepileptics * Spasmolytics or skeletal muscle relaxant * Neurotropic vitamin therapy-- Contraindicated 1n case of underlying malignancies * Appropriate management for cases with oncologic etiologyNeurology Pre red by Group 433 (2007) 55) Progressive muscular dystrophies. Actiology, pathogenesis, cli treatment. ical features and - Characterized by muscle fiber necrosis, regeneration, fibrosis and replacement with fatty tissue. - Usually associated with abnormalities in dystrophin associated glycoprotein complex. _ [Duchenne dystrophy _ cker Dystro| Pathogenesis |~ Gene for dystrophin is located at | Deletion within central rod domain of | Xp2.1 gene at Xp2.1 | - Point mutation and deletion L affecting the terminal domains _ Clinical = Male birth ; 1-3 y.0 - Presenting at later age (10- features - Delayed motor development 13y.0.)with limb girdle - Scoliosis, contracture, loss of | involvement and ambulation at 12 y.o, | pseudohypertrophy - Pseudohypertrophy of muscle at calf |- May involve cardiac system = Pelvic muscle weakness (Gower | sign, cannot climb stair and rise | |__ from chair) te investigation - Gene testing Creatine kinase (CK): Substantially {(several thousands times) ECG,electromyography _ Facioscapulohumeral(FSH)_ Etiology: Autosomal dominant disorder. Pathogenesis: Unknown Clinical features - Facial weakness (mild or asymmetrical) - Periscapular weakness (winging of scapula, rising up of scapula on abduction) ~ Weakness of the humeral muscle ~ Proximal lower limb pattern of weakness —+ dromedary/camel backed gait - Pseudohypertrophy & cardiac involvement (x a feature) - Tfrequency sensorineural hearing loss and exudative retinal telangiectasis complicate some early cases (Coat’s syndrome) 119Neurology Prepared by Group 433 (2 Etiology: Autosomal dominal multisystem disorder Pathogenesis. Unstable trinucleotide repeat expansion j Characterized by y myotonia (failure of immediate n Clinical features - Frontal baldness ~ Myopathic face with ptosis ~ Jaw hanging ~ Wasting muscle of mastication — hollow temporal fossa and cheeks ~ Wasting of neck and shoulder muscle | Cataract - Disorders of smooth muscle: © Gut motility disorder © Constipation * Poor bladder emptying ~ Cardiac disease: dilated cardiomyopathy and atrio-ventricylar block - Respiratory failure due to: * Intercostals and diaphragmatic weakness ®° Impaired swallowing with risk of aspiration and central sleep apnoea ~ Diabetes due to insulin resistance - Testicular atrophy and subfertility ‘Treatment of all type of muscle dystro; mplomatic treatment: ~ Surgery for correction of scoliosis and cataract ~ Active central of contracture and non-invasive ventilation ~ Sodium blocker: Phenytoin, procainamide and ete ~ Pacemaker device - Genetic counseling 120Neurology Prepared by Group 433 (2007) 56) Myasthenia Gravis. Etiology, pathogenesis, clinical features and treatment. - Isa disorder of neuromuscular transmission characterized by: + Weakness and fatiguing of some or al] muscle groups; + Weakness worsening on sustained or repeated exertion, or towards the end of the day relieved by rest. ~ Usually among women with onset between 30-40 years old, ‘tiology. 5 consequence of an autoimmune destruction of acetylcholine receptors Pathogenesis ~ Abnormal function of thymus gland after inflammatory process. ~ Acetylcholine receptor antibodies bind to receptor —+ destroy receptor ~ Synthesis of new receptors is disturbed. ~ At first it produces focal oculomotor syndrome, then gradually affecting fascial and oral muscles and possible generalized affecting all limbs and cervical muscle. Clinical features ~ General fast fatigue of muscles and worsening towards the end of the day - Oculomotor symptoms: Ptosis, diplopia & muscle paresis ~ Patient cannot gaze or focus for a long time - Hyporeflexia - Bulbar syndrome (dysphonia, dysphagia, dysarthria, regurgitation, nasolalia) - A lot of saliva, weakness of tongue muscle and jaw ~> speech disorder - Weakness of facial muscle — expressionless - Aspiration pneumonia as a complication Differential Diagnosis - CNH Hesion © « improve after rest » May V some other signs eg. Accommodation, miosis etc Investigation 1) Pharmacological: Anticholinesterase drug is used eg. Tensilon {edrophonium}; short action: 2-4min; IV; 2-10mg slowly 2) Serological: Detection of acetylcholine receptor Ab (~-90% of patient) 3) Electrophysiological 4) Chest x-ray & CT of chest 121Neurology Prepared by Group 433 (29 Treatment 1) _Anticholinesterase drug _ Drug Duratis (Edrophonium Neostigmine Pyridostigmine + Mcchotinoblocker (eg, Atropine) may be requited to counter $.15, (nausea, vomiting, diarrhe muscle fasciculation, {weukness) ~ Anticholinesterases rarely give complete symptomatic relief & large doses > Choline crisis 2) Steroids (Prednisolone 60mg/day) 3) Immunosuppressants (Azathioprine & cyclosporine) 4) ThymectomyNeurology Prepared by Group 433 (2 57) Epilepsy. Etiology, pathogenesis, clinical and treatment Epilepsy Chronic brain disease characterized by repeated, stereotyped and uncontrolled seizures. or epileptic attacks, which is the consequence of a paroxysmal discharge of brain neurons Etiology = Idiopathic epilepsy (family history, childhood or adolescent) - Symptomatic epilepsy - Cryptogenic epilepsy (unknown cause) Causes of symptomatic epilepsy Head trauma Birth trauma High fever Biochem imbalance _ Circulatory disturbance Pathogenesis - Abnormal electrical act in brain. - Central role for the excitatory neurotransmitter glutamate, which produces depolarization shift by activating receptors which in turn facilitate cellular influx of Na, K and Ca. - GABA has inhibitory influence in containing abnormal cortical discharge and prevent the development of generalized seizures. International classification of epileptic seizures PARTIAL (focal/local) seizures (80% of adult epilepsies) © Simple partial seizures (safe conscious): © Motor o Sensory * Complex partial seizures (when partial seizures is accompanied by impaired consciousness) GENERALIZED ° Absences * Tonic/clonic seizures * Myoclonic seizures * Atonic seizures UNCLASSIFIED eg. Neonatal seizures Clinical Features 1) Aura: Symptoms immediately before a seizure and will localize the attack to the brain origin. 2) Ietal peiod: Include the epileptic seizure 3) Postictal period: Time immediately after the attack during when the patient may be confused, disoriented.Neuratogy Prepared by Group 433 (00 Tonic/elonie attacks (Grand mal) (i) Tonic phase (10- seconds) * Loss of consciousness, fall to ground * Eyes open, pupils dilated, elbow flexed, legs extended, teeth clenched, breath evanosis * Bowel/bladder control may be lost at end of phase ii) Clonie phase (1-2 minutes) * ‘Tremor to violent general shaking + Eyes roll backwards, tongue may be bitten + Tachycardia, breathing recommences at end of phase ~The patient then sleeps and cannot be roused. On regaining consciousness, confusion headache are present ~ He may feel weakness for hours or even days, muscle may ache as result of violent moveme May be weak for 24-48 houts after seizure —» Todd’ paralysis, ~ Trauma occurs frequently, either as a result of fall Absences (Petit mal = Onset usually in childhood (byw 4212 y.o.) - Absence may ocSuF many times a day for 5-15 seconds, ~ Child may stop talking in mild sentence of cease walking and may resume a few seconds latex ~ After attack, full recovery of consciousness, no confusion and no memory of the seizure. Myoclonic seizures ~ Sudden, brief generalized muscle contraction, often occur in the morning-and are occasio’ associated with tonic/clonic seizures, ~ May also occur in degenerative and metabolic diseas Alonic seizures Characterized by Joss of muscle tone and sudden fall, consciousness may only be lost briefly. Simple motor seizures (Jacksonian) ~ These arise in the frontal motor cortex with movement occurring in contralateral fice, trunk © limbs, ~ Consist of a march of involuntary movement from one muscle group to another. ~ Motor seizures can pass in generalized seizures Adversive seizures ‘The patients eyes and head turn away from the site of focal origin in the motor cortex of the frontal lobe (frontal “gaze center”). Simple sensory seizures ~ These arise in the post-Rolandic sensory cortex. ~ Consist of paraesthesia or tingling in an extremity or on the face. - A march similar to the Jacksonian motor seizure may occur - Sensory seizure can pass in motor and generalized seizuresNeurology Prepared by Group 4 (2007) Complex partial seizure Attack usually originate within the temporal lobe and characterized by a complex aura (initial symptom), some impairment of consciousness and following symptoms: —+ Memory, Motor, Affective, Visceral disturbances, Automatism Treatment - Drug treatment should be simple (monotherapy), continuously - If patient goes 3 years without an attack, withdrawal of therapy should be considered. - Medicines: * Sodium Valproat. line drug of generalized epilepsies, absences. myoclonic seizures ‘© Carbamazepine + \" line drug of any partial seizures 4 Ethosuximide (in absences) in childhood uusness * return b/w attacks > Status epilepticus, May be life threatening with the development of pyrexia, coma & circulatory collapse. May occur with © Head injury On reducing drug therapy Alcohol, drug intoxication Infectious, metabolic disturbances (hyponatremia) Pregnancy eo0c Treatment General ¢ © Establish an airway - O; inhalation 10L/min TV infusion : 500mL 5% glucose /0.9% NaCl Vital signs recorded regularly Prevent hyperthermia recific © Diazepam + Phenytoin © Seas x we sophed » wamigsne ZECSBOST oy YQ YS =p treokmects : 125