Steps to a Neurologic Diagnosis Steps to a Neurologic Diagnosis “The following outline summarizes the steps for artiving at a neurologic diagnosis with the use of the aids in this book: 1. The neurologic examination 2, Are the neurologic findings real or of localizing significance? 3. Where is the lesion responsible for the neurologic signs? ‘A. What is the anatomic level of the lesion? B. Which tracts or nuclei are involved? 4. What is the lesion? ‘A. Does the patient have a “treatable” disease? B. Does the patient have a space-occupying lesion? C. The value of a good history D. Which diagnostic procedures confirm the diagnosis? 1. THE NEUROLOGIC EXAMINATION ‘The finer technique of the neurologic examination have been discussed repeatedly in many standard neurologic texts and handbooks; there- fore, they are not elaborated upon here. Nevertheless, the routine neurologic examination is illustrated below to simplify the recall pro- ‘cess. Once the routine examination is completed, the clinician may ‘wish to substantiate any abnormal findings by further tests of the same tract, nucleus, or region. These tests are indexed with the respective anatomie structures in Appendix A. 2, ARE THE NEUROLOGIC FINDINGS REAL OR OF LOCALIZING SIGNIFICANCE? Special features of conversion hysteria are listed in Table 1-1. This disorder is 2 common cause of curious neurologic findings and often can be diagnosed by a careful examination. Frequently, “abnormal” findings are caused by associated metabolic disease (e.g., hyper- thyroidism, drug-induced disease, and so on), increased intracranial pressure, or poor cooperation of the patient with low mentality, emo- tional instability, or subnormal levels of consciousness. Perhaps the findings are consistent with the patient's age. On the other hand, the patient's efforts to conceal his disabilities may yield normal examina- tion. The clinician should not neglect these aspects of interpretation.4 The Diagnostic Interpretation of Neurologic Signs TABLE 1-1. EXAMPLES OF FINDINGS SUGGESTIVE OF HYSTERIA" Midline anesthesia or analgesia Inconstant pattern of sensory loss Tuning-fork vibrations not felt on one side of skull Complete loss of touch and pain sense without loss of temperature or position Complete loss of position and vibratory sense in leg but ability to walk normally Sensory loss not in conformity with an anatomic distribution Complete blindness with pupils that react to light (except in bilateral occipital lobe lesions; EEG helpful) Tubular visual fields that remain the same size, regardless of distance from examining chart Paralyzed arm and hand not in the typical hemiplegic postion (flexion of el- ‘bow, pronation of forearm, flexion of fingers only at interphalangeal joints) Hemipiegia without greater distal paralysis Tests for muscle power showing perhaps far greater disability than the perfor- ‘mance in other tests would suggest e., walking, coordination, and so forth) Objective parts of examination, such as optic fundi, reflexes normal Hoover's sign (attempt by patient to raise paralyzed leg without thrusing nor- ‘mal leg downward) ‘Adequate power in limbs in recumbent examination but complete inzbilty to walk or stand (astasia—abasia) Grand mal seizures without micturition, cyanosis, salivation, orbiting o tongue = Note, nane ofthe above features x diagnostic of hysteria; nor can they exclude organic ease Because both may oceur together iis " ae 3. WHERE IS THE LESION RESPONSIBLE FOR THE NEUROLOGIC SIGNS? ‘The location of the lesion tells the surgeon where to operate. More important to the clinician, itis the key to a differential diagnosis. This process involves two phases: (1) location on the longitudinal plane (i.e., the anatomic level) and (2) location on the transverse plane (i.e., the tracts or the nuclei involved). In addition to the tables, the two basic neuroanatomic drawings on pages 69 and 119 will assist the reader in this task. Each portrays longitudinal and trancverse views of the ner- vous system, WHAT IS THE ANATOMIC LEVEL OF THE LESION? For proper application of the diagnostic tables in this book, first it must be determined whether the lesion is above or below the foramen mag- num. Asa rule, if there are no cranial nerve signs, no papilledema, no nystagmus, no mental or speech changes, and no seizure disorders, the lesion is below the foramen magnum. However, there are exceptions, for example, parasagittal lesions and early cerebellar lesions. Above theSteps to a Neurologic Diagnosis TABLE ‘MEDULLA Signs of involvement of one or more ofthe following cranial nerves: V, VI, IX, X, XI, XI (see Table 1-5, Section C) Vertical and/or horizontal nystagmus Heriplegia, paraplegia, quadriplegia Crossed hemianesthesia andior hemianalgesia (may not be present above neck) Dysmetria, dysdiadochokinesia, dyssynergia, intention tremor (usually unilat- eral) ‘Wide-based ataxic gait with falling to one side PONS Signs of involvement of one or more of cranial nerves V, VI, Vil Signs of upper metor neuron involvement of one or more of cranial nerves X, XI, XI Paralysis of conjugate lateral gaze Hemiplegia, paraplegia, or quadriplegia Crossed hemianestresia and/or hemianalgesia Internuclear ophthalmoplegia MIDBRAIN. Signs of involvement of cranial nerves Ill or IV (infrequently Parinaud’s syn. drome) Signs of upper motor neuron involvement of one or more of cranial nerves V, VIL, X, XI, Xi Hemiplegia, paraplegia, or quadriplegia Hemianesthesia and/or hemianalgesia Dysmetria, dysdiadochokinesia, dyssynergia, intention tremor (usually unilat eral) Head and posture tilt, wide-based ataxic gait DIENCEPHALON Rarely visual field cuts Signs of upper motor neuron involvement of one or more of cranial nerves V, Vil X, XL XI Hemiplegia (usually global) or quadriplegia Hemianesthesia ardior hemianalgesia Fleeting deep pains in face, arms, andlor legs (thalamus) “Tremor, rigidity, chorea, athetosis ‘One oF more of te signs at each Tevel may not be present in the individual case Basiesly, the sigs sre some combination of canal nerve and long tract involvement but ‘ether may occur alone. The specic level of cranial nene involvement determines the level ofthe lesion -LINICAL KEY TO THE LEVEL OF BRAIN-STEM INVOLVEMENT* foramen magnum the lesion can be localized further to respective levels of the brain stem (e.g., medulla, pons, and so forth; see Table 1-2) if there are cranial nerve signs (excluding supranuclear palsies). The lesion can be localized to the cerebrum if there are overt mental or seizure disorders (excluding petit mal). .“The Diagnostic Interpretation of Neurologic Signs TABLE 1-3. DETERMINATION OF ANATOMIC LEVEL OF LESION BY CLINICAL LEVEL OF PARALYSIS OR ANESTHESIA? Clinical Level able Anatomic Level of of Involvement Single or Space-Ocrupying Lesion ‘ower extremity Below T? in cod exception in parasagital lesions) Lumbosacral roots and ples Upper estemity slone Conical roots (eg, cervical disk) Brachial ples (eg, scalewus anticus syndrome) Rarely peripheral ceviealrenes (@, apal tun nel syndrome, traumatic neuroma) Early intramedullary cervial cord lesion Al four extemits Cenieal cord ‘exception in parasagital lesions) Head alone (cranial nerve signs) Peripheral potion of cranial nerves (enteral ‘carlid aneurysm, pitty adenoma, cerebello- pontine ange tumen) Eatly intramedullary iavolvement of brain stem (eg. braie-ster glioma, pinealoma) Head, upper andor lower cerebrum fexttemity Brain stom (eg, advanced brainstem glioma) 5 abe dl ony wi sil esos. The location of mull sions ao posi In the peripheral nerves, st the myoneural junction, and in muscle at any level Generally, the lesion in neurologic disorders causes a “clinical level” corresponding to the anatomic level of the lesion. Again a notable exception to this principle occurs in lesions anywhere along the “axis of the cerebellar system. Table 1-3 and the figures on pages 28 to 33 correlate the clinical and the anatomic levels in various lesions of the sensory and the motor pathways.” Signs explainable by a lesion at one level arouse suspicion of space-occupying lesions (e.g., neoplasm, hematoma, abscess, and others), the most urgent conditions in the differential diagnosis (see discussion below on Does the Patient Have a Space-Occurying Lesion?). If the findings will not fit @ lesion at one level, the clinician should perhaps consider a disease characterized by multiple lesions, for example, multiple sclerosis or neurosyphils. WHICH TRACTS OR NUCLEI ARF INVOLVEN? In Tables 1-4 and 1-5 the abnormal findings are categorized according to the tract, nucleus, or the region destroyed. If the anatomic level is below the foramen magnum, Table 1-4 applies. Ifthe anatomic level is above the foramen magnum, Table 1-5 applies. For the initial step, select the tracts, the nuclei, or the regions that best explain the * When studying the fgutes, keepin mind thatthe posterior andthe lateral columns cross in the upper cord and the low brain stem, while bers ‘hat contribute to the spinothalamic frets cross near their level of origin. ' Rarely muliple lesions are explained by two of mare conditions, one of which may bea space-occupying lesion,Steps to a Neurologic Diagnosis TABLE 1-4. CLINICAL KEY TO THE INVOLVEMENT BELOW THE FORAMEN MAGNUM* POSTERIOR COLUMN Numbness and tingling Loss of vibratory sense Loss of touch and position sense Dysmetria LATERAL COLUMN, Weakness (diffuse, but involves distal muscles more) Loss of voluntary movement Increased deep tendon reflexes Ankle, patellar, wrist clonus Babinski’, Oppenheim’s, Chad- dock’s, “Hoffmann’s, Tromners signs SPINOCEREBELLAR TRACTS. Usually no signs at all unless there 's associated involvement of the cerebellum (see Cerebellar LATERAL SPINOTHALAMIC TRACT Contralateral changes VENTRAL SPINOTHALAMIC TRACT Contralateral changes VENTRAL COMMISSURE Bilateral changes SENSORY ROOT Radicular pain (Laségue's and ‘Minor’s signs) Numbness and tingling ase of vibratory and postion Loss of touch, temperature, and pain sense in segmental fashion ‘Absent deep tendon reflexes with- MOTOR HORN OR ROOT Weakness (confined to individual muscles) Loss of deep tendon reflexes ‘Muscular atrophy (more selective) Fasciculations Reaction of degeneration Flaccid muscles Loss of Ueep tendon reflexes ‘tania accentuated by closing eyes Romberg’s sign present Flaccid neurogenic bladder Loss of superficial abdominal and and cremasterc reflexes Muscle spasticity Spastic gait Spastic neurogenic bladder (can be flaccid in acute lesions) Hemispheres in Table 1-5, Sec: tion 8) Loss of pain and temperature sense Loss of superficial touch sense (rarely) Loss of pain and temperature sense ‘out atrophy or reaction of de- ‘generation Flaccid muscles “Trophic changes Broad-based ataxic gait accentu- ated by closing eyes Flaccid neurogenic bladder if $2, 33, oF $4 is involved Loss of anal reflex and flaccid neu- rogenic bladder if $2, $3, or $4 is invohed No pathologic reflexes \Vasomotor changes (if lateral horn is involved)The Diagnostic Interpretation of Neurologic Signs TABLE 1-4—(Continued) PERIPHERAL NERVE ‘Weakness, paresthesia, pain ‘Absent deep tendon reflexes Tenderness of nerves and muscles ‘Muscular atrophy Nonsegmental combined sensory Fibrillaions and reaction of de- loss (e.g, glove and stocking) generation Los of vibratory and postion Trophic and vasomotor changes sense Steppage gait ‘SYMPATHETIC GANGLION Horner's syndrome \Vasomotor ckanges (ie, dermo- Anhidrosis ‘raphia) Hypertension MUSCLES, ‘Weakness (often proximal more No fasciculatons or reaction of than distal degeneration ‘Atrophy (but occasional “hyper- Deep tendor reflexes retained trophy” until late (out diminished) ‘Waddling gait ~T lane ae pomolieal nie otbenrse wpedied, One or more sgns under each suc- ture may be absent minivdual case Losses may be lol © paral neurologic signs in each case. Then, for a differential diagnosis of the principal diseases involving each tract, nucleus, of region refer to Table 1-6 (below the foramen magnum) or Table 1-7 (above the foramen magnum). By locating the lesion on the two master illustrations, the clinician can make a comparison with the lesions on the plates of each disease in the differential. As a further aid to diagnosis, case reports summarizing the most typical clinical features of each disorder accompany each plate, The following clinical case illustrates the steps taken in diagnosis to this point: A 30-year-old white female complained of difficulty in walking and of ‘numbness and tingling in all four extremities for the past 6 months. Exam- ination revealed increased patellar and Achilles reflexes, bilateral Babinski's signs, loss of position and vibratory sense in both lower extremities, and a spastic-ataxic gat. Because there are no cranial nerve signs, papilledema, and so forth, in this case, a lesion would be suspected below the foramen magnum. ‘Table 1-4 indicates that involvement of the posterior and the lateral columns can cause these signs. Appendix A suggests that checking the patient further for graphesthesia, two-point sensiblity, ankle clonus, and so on would confirm the signs of posterior and lateral column disease, In Table 1-6 and the color plates a differential diagnosis ofSteps to a Neurologic Diagnosis ‘TABLE 1-5, CLINICAL KEY TO INVOLVEMENT ABOVE THE ‘FORAMEN MAGNUM SECTION A: Major Tracts of the Brain Stem* PYRAMIDAL TRACT Upper motor neuron involvement Loss of superficial abdominal and ‘of one or more cranial nerves cremasterc reflexes Y, MIL, X, XL, XI Muscle spasticity Hemiplegia, quadriplegia Spastic gait Babinsk’s, Oppenheim’s, Chad Spastic neurogenic bladder dock’s, Hoffmann’s signs MEDIAL LEMNISCUS Numbness and tingling Dysmetria Loss of vibratory sense ‘Ataxia accentuated by closing eyes Loss of deep touch and position Romberg's sign RESTIFORM BODY OR BRACHIUM CONJUNCTIVUM Homolateral changes Hypotonia Incoordinated movements of ex- Decreased deep tendon reflexes teemities (rarely) Dysdiadochokinesia Head tilt and posturing (occa Dyssynergia sionally) Dysmetria often with intention Reeling, broad-based gait wemor SPINOTHALAMIC TRACTS Loss of pain and temperature sense Loss. of superficial touch sense (carely) SECTION 8: Cerebeum, Extrapyramidal System, and Cerebellum FRONTAL 10RE Headache; focal, jacksonian, or jocularity; response to questions ‘generalized motor seizures with silly answers) Expressive aphasia Hemiplegia or monoplegia (usu- Confusion ally with central facial palsy Disorientation in time, space, and ‘and often palatal or lingual person weakness) ‘Motor apraxia Grasp, after-gaasp, and sucking re- Witzelsucht syndrome (excessive flexes PARIETAL LOBE Headache Loss of graphesthesia Jacksonian sensory seizures Loss of two-point sensibility Loss of vibratory and position ——_Astereognosis sense Ideational and ideomotor apraxia10 The Diagnostic interpretation of Neurologic Signs TABLE 1-5—(Continued) ‘TEMPORAL LOBE Headache Uncinate fits (unusual tastes, smells, hallucinations, déja vu [already seen), psychomotor ac tivity) OcciPrTAL LORE ‘Visual auras and seizures (flashes flight, blindness) Homonymous hemianopsia, fe. PARIETAL OCCIPITAL JUNCTION Visual perceptive aphasia Nominal aphasia Gerstmann’s syndrome: inability to differentiate right from left EXTRAPYRAMIDAL SYSTEM Emotional instability (eg., uncon- twollable outbursts of laughter) Masked facies Myerson’s sign ‘Monotonous speech Fixed head and trunk Muscular rigidity (cogwheel 0 plastic) (CEREBELLAR HEMISPHERES Homolateral changes Syllabie or staccato speech Nystagmus Incoordinated movements of ex: tremities Dysciadochokinesia Dyssynergia VERMIS OF CEREBELLUM Rocking forward and backward in Romberg position Auditory perceptive aphasia Homonymous hemianopsia or ‘quadranopsia ‘quently with macular sparing or incongruity Difficulty in performing simple arithmetic Difficulty in diferentating one finger from another Involuntary tremor at rest (but dis- appearing during sleep) Athetosis Chorea Hemiballism Propulsion and retropulsion Grotesque, clownish, or shor stepped gait Dysmetria Intention tremors Hypotonia Decreased deep tendon reflexes Head tilt and posturing occa: sionally) Reeling, broad-based gait Trunk ataxia SECTION C: Cranial Nerves 1. OLFACTORY NERVE Loss of sense of smell 1. OPTIC NERVE ledema