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NEUROLOGY

YEAR 4 SUMMER CRASH COURSE


THIRD SESSION
• Epilepsy (ACE)
• Guillian-Barre syndrome
• Hyperkinetic movement disorders
• Hypokinetic movement disorders
• Metabolic neuropathy (B12 and
CDM diabetes)
• MND
CONDITIONS • MS
• Parkinson’s (ACE)
• Spinal cord compression
(degenration, tumor)
• Stroke and TIA (ACE)
1) GUILLIAN BARRE SYNDROME
Not commonly
tested in MOSLER
EXAM TIP:
GBS TYPES Remember age of
presentation
15-35, 50-75
AUTOIMMU
NE,
DEMYELINA • Acute Inflammatory Demyelinating Polyneuropathy
(ADIP)
TING, • Acute Motor Axonal Neuropathy (AMAN)
TriggeredPNS
ACUTE, by • Acute Motor and Sensory Axonal Neuropathy
• Miller-Fischer Syndrome (MFS)
INFECTION
(campylo MILLER-FISCHER

jejuni) in past SYNDROME


• TRIAD of ATAXIA,

6 weeks AREFLEXIA,
OPTHALMOPLEGIA
• DESCENDING
CLINICAL
FEATURES
• Tingling and numbness preceded by
weakness, starting from legs going
upwards
• Reduced sensation in affected areas
• Ataxia, unsteady walk
• Autonomic dysfunction : urinary
retention, postural htn, tachycardia, htn
• Respiratory distress
• Facial weakness, speech problems
INVESTIGATIONS AND
MANAGEMENT
• Nerve conduction studies
• Routine blood tests
• MRI brain and spine
• Anti-ganglioside
• LP – albuminocytologic dissociation

MANAGEMENT
• First line : IV Ig, Plasma exchange
• Additionally : Thromboprophylaxis, Physiotherapy, ICU
HYPOKINETIC MOVEMENT DISORDERS
1. Parkinson’s disease (Idiopathic Parkinsonism)
2. Multiple Systems Atrophy (MSA) – autonomic dysfunction, cerebellar and parkinsonism
3. Progressive Supranuclear Palsy (PSP) – early falls, frontal cognitive issues, vertical gaze
palsy
4. Dementia with Lewy Bodies – memory problems, somnolence, hallucinations, later
parkinsoian symptoms
5. Corticobasal syndrome – mutism, alien limb syndrome
6. Drug induced
7. Vascular
8. Metabolic
MOSLER TIP:
Remember for
2) PARKINSON’S DISEASE
Pathophysiology
Neurodegenerative

Loss of dopaminergic neurons in the substantia nigra par in


basal ganglia, accumulation of lewy bodies
Loss of transmission between basal ganglia, thalamus,
motor cortex
Results in loss of control of voluntary movements

Idiopathic

Clinically diagnosed
Exam tip:
Remember the
•DIAGNOSTIC CRITERIA:

CLINICAL
symptoms well to
• BRADYKINESIA and any one of diagnose
• Cogwheel rigidity Common CDF case

FEATURES • Resting tremor


• Postural instability

MOTOR SYMPTOMS NON-MOTOR


SYMPTOMS
• Shuffling gait, reduced • REM sleep
arm swing behaviour disorder
• Pill rolling (resting) • Restless legs
• Lateral lean, syndrome
camptocomia • Autonomic
• Falls and imbalance symptoms
• Memory issues
• Depression and
anxiety
• Hypersalivation
• Mask like face
MANAGEMENT

Motor symptoms affecting quality of life Motor symptoms not affecting quality of life

•Levodopa + decarboxylase inhibitor (Co- A choice of one of the following:


beneldopa) 1. Dopamine agonist (non-ergot derived)
Pramipexole, ropinirole , Ergot derived
medications (e.g. bromocriptine) should be
avoided as they are associated with cardiac and
pulmonary fibrosis
2. Monoamine oxidase B inhibitor (Selegiline,
rasagiline)
• COMT inhibtors (entacapone), 3. Levodopa + decarboxylase inhibitor (Co-
beneldopa)
preserve levodopa
• Amantadine (increase release of
dopamine)
• Apomorphine infusion
• DBS
3) B12
NEUROPATHY
Pathophysiology
In B12 def, propionyl CoA replaces Methylmalonyl CoA and causes
Demyelination
Clinical features and Complications
• Anemic symptoms, glossitis, angular stomatitis
• Tingling, weakness, fatigue
• SACD : mixed sensory and UMN signs
• PN : absent ankle jerk, brisk knee jerk, upgoing plantars
• Optic neuropathy
• Neuropsychiatric symptoms : depression, psychosis, dementia
• Restless leg syn, paraplegia
• Bowel/bladder complications
EXAM TIP:

INVESTIGATIONS and
Remember what investigations
show and was tested in wriske

MANAGEMENT
• FBC : macrocytic anemia
• Blood film : Megaloblasts
• Vit B 12 levels in blood : normal is approximately above >200 picomol/

MANAGEMENT
• Dietary advice : more eggs, diary, meat
• IM hydroxocobalamin injection initially 3x a week for 2 weeks thereafter
1. If pernicious every 2-3 months for life IM
2. Not diet related then oral tablets 50-150mcg daily / 2x yearly 1mg injection
4) MOTOR
NEURON
DISEASE
RISK FACTORS
• Old age

MND TYPES • Male


• FH

1. Amyotrophic lateral sclerosis:


• The familial form is associated with mutations in superoxide dismutase (SOD1)
• UMN signs: predominantly affects the corticospinal tracts
• LMN signs: also affects anterior horn cells
• Overall, mixed UMN/LMN signs may be seen

2. Progressive muscular atrophy:


• LMN signs: predominantly affects the anterior horn cells
• Best prognosis

3. Primary lateral sclerosis:


• UMN signs: predominantly affects the corticospinal tracts

4. Progressive bulbar palsy:


• Affects the suprabulbar nuclei and cranial nerves, producing speech and swallow issues
• Worst prognosis
CLINICAL
FEATURES
• Progressive weakness, Falls
• Mixed UMN and LMN signs
• Tongue fasciculations
• Speech, swallow difficulties, dysarthria,
dysphagia
• Respiratory difficulties
INVESTIGATIONS and
MANAGEMENT
• Electromyography
• Nerve conduction studies
• MRI spine
• Pulmonary function tests

MANAGEMENT
• Riluzole (redcuces progression)
5) MULTIPLE
SCLEROSIS

• Autoimmune
• Demyelination of
CNS
EXAM TIP
Common

Risk factors MOSLER station

• Age 20-40
• Female
• EBV
• Vit D def
• Family History
Patterns of disease

1. Relapsing Remitting : most


common, can progress to secondary
MS
2. Secondary progressive : gets worse
with no remission
3. Primary progressive : progressively
worse, no remission, common in
OLDER patients
CLINICAL
FEATURES
• Optic neuritis : blurry vision, painful
• Internuclear Opthalmoplegia
• Sensory : numbness, tingling, sensation
loss, fatigue
• UMN : ataxia, tremor
• Bowel, bladder dysfunction
• Uhtoff’s and Lhermitte’s phenomenon
INVESTIGATIONS and MANAGEMENT
• MRI brain and spine : demyelinating plaques
• LP : Oligocloncal bands
MANAGEMENT
• RELAPSE : steroids, plasma exchange
• MAINTENANCE : Beta-interferon, Monoclonal antibodies,
Glatiramer acetate, Fingolimod
6) CAUDA
EQUINA
SYNDROME
DEFINITION AND CAUSES
• Lumboscral nerve roots that extend below L1/2 are compressed
• Important to explore in patients presenting with new/worsening lower
back pain
• MOST COMMON CAUSE : central disc prolapse L4/5 or L5/S1
• Tumors : primary/ metastatic
• Infection : abscess, discitis
• Trauma
• Haematoma
CLINICAL PRESENTATION
• Suspected Cauda Equina Syndrome:
1. bilateral sciatica pain
2. leg weakness
3. saddle paraesthesia
4. bladder disturbance and bowel disturbance.
• Complete Cauda Equina Syndrome:
1. Urinary retention is present
2. Overflow urinary incontinence may be present.
3. Loss of anal tone and peri-anal sensation.
4. Faecal incontinence is a late sign of advanced disease.
5. Saddle paraesthesia is more widespread and complete.
• Incomplete Cauda Equina Syndrome:
1. Urinary retention is not present.
2. Subjective reduced urination sensation
3. difficulty passing urine, poor stream, reduced urge to void bladder.
4. Anal tone and peri-anal sensation might be normal.
5. Saddle anaesthesia may be present in a more patchy/unilateral way.
INVESTIGATIONS and
MANAGEMENT
1. DRE
2. POST-VOID BLADDER SCAN
3. URINE DIPSTICK
4. MRI SPINE (GOLD STANDARD)

• MANAGEMENT : Surgical decompression


COMPLETE VS INCOMPLETE SCC
Pattern Features

Complete spinal cord injury


• All motor and sensory function below the SCI level
• E.g. high cervical cord level - quadriplegia, respiratory insufficiency, loss of bladder and
bowel function, neurogenic shock

Central cord syndrome


• Usually involves cervical spine
• Weakness in upper extremities > weakness in lower extremities
• Variable sensory loss below the level of injury
• Most common pattern of incomplete SCI

Anterior cord syndrome


• Disruption of anterior spinal cord or anterior spinal artery
• Loss of motor function below the level
• Loss of pain and temperature sensation (anterior column)
• Preservation of fine touch and proprioception (posterior column)

Posterior cord syndrome


• Disruption of posterior spinal cord or posterior spinal artery (rare)
• Loss of fine touch and proprioception (posterior column)
• Preservation of pain and temperature sensation (anterior column)

Brown-Sequard syndrome
• Hemisection lesion of the spinal cord
• Unilateral spastic paralysis
• Ipsilateral loss of vibration and proprioception
• Contralateral loss of pain and temperature sensation
7) ISCHEMIC
STROKE

COMMON
MOSLER
OXFORD STROKE CLASSIFICATION
Stroke type Notes

• DIAGNOSIS CRITERIA Total anterior circulation infarcts


(TACI, c. 15%)
• involves middle and anterior cerebral arteries
• all 3 of the above criteria are present

1. Unilateral hemiparesis, sensory loss


Partial anterior circulation infarcts
(PACI, c. 25%)
• involves smaller arteries of anterior circulation e.g.
upper or lower division of middle cerebral artery
2. Homonymous hemianopia • 2 of the above criteria are present

3. Higher cognitive dysfunction (dysphasia)


Lacunar infarcts (LACI, c. 25%)
• involves perforating arteries around the internal
capsule, thalamus and basal ganglia
• presents with 1 of the following:
• 1. unilateral weakness (and/or sensory deficit) of
face and arm, arm and leg or all three.
• 2. pure sensory stroke.
• 3. ataxic hemiparesis

Posterior circulation infarcts (POCI, c.


25%)
• involves vertebrobasilar arteries EXAM
• presents with 1 of the following:
• 1. cerebellar or brainstem syndromes TIP:
• 2. loss of consciousness
• 3. isolated homonymous hemianopia Headache
is more
common in
LOCALISATION OF STROKE SITE
Site of the lesion Associated effects
Anterior cerebral artery Contralateral hemiparesis and sensory loss, Midbrain: WEBER syndrome Pons: LOCKED-IN syndrome Medullary: WELLENBERG/LATERAL
lower extremity > upper

Middle cerebral artery Contralateral hemiparesis and sensory loss,


upper extremity > lower • Ipsilateral 3rd • Preserved cognition • Contralateral loss of pain
Contralateral homonymous hemianopia nerve palsy but no movement or and temp in body
Aphasia • Contralateral communication due • Ipsilateral loss of pain
hemiparesis to paralysis of and temp in face
Posterior cerebral artery Contralateral homonymous hemianopia with voluntary muscles • Ipsilateral ataxia,
macular sparing nystagmus, N/V, vertigo
Visual agnosia • Ipsilateral hoarseness,
horner's syndrome
Weber's syndrome (branches of the posterior cerebral Ipsilateral CN III palsy
artery that supply the midbrain) Contralateral weakness of upper and lower
extremity

Posterior inferior cerebellar artery (lateral medullary Ipsilateral: facial pain and temperature loss
syndrome, Wallenberg syndrome) Contralateral: limb/torso pain and
temperature loss
Ataxia, nystagmus

Anterior inferior cerebellar artery (lateral pontine Symptoms are similar to Wallenberg's (see
syndrome) above), but:
Ipsilateral: facial paralysis and deafness

Retinal/ophthalmic artery Amaurosis fugax


Basilar artery 'Locked-in' syndrome
INVESTIGATIONS and
MANAGEMENT
• FAST and ROSIER score
• Non contrast CT (exclude hemorrhage and start thrombolysis)
• MRI
QUESTION 1
A 30-year-old man presents to the Emergency Department with a complaint of progressively worsening
weakness in his lower limbs. He mentions that this weakness started a week after recovering from an episode of
gastroenteritis. On examination, you notice bilateral foot drop and decreased reflexes in his lower limbs. The
patient denies any sensory disturbances.

What condition is most likely responsible for the patient's symptoms?

A)Amyotrophic lateral sclerosis (ALS)


B) Guillain-Barré syndrome
C) Multiple sclerosis
D) Myasthenia gravis
E) Parkinson's disease
QUESTION 2
A 19-year-old man presents to the Emergency Department stating that he has had a stroke.

When asked about his symptoms, he explains that he was sitting at work 2 hours ago when he suddenly had the
sensation of smelling roses. He recalls asking his colleagues where the smell was coming from, but none of them could
smell it. He states that he also felt a little sweaty at the time. The episode lasted for a couple of minutes and then the
smell disappeared. He has a history of headaches but is otherwise well.

What is the most likely diagnosis?


A) Complex focal seizure
B) Factitious disorder
C) Focal aware seizure
D) Migraine with aura
E) Transient ischaemic accident
QUESTION 3
• An 82-year-old man with advanced Parkinson's disease is seen in the emergency department with a UTI. His
summary care record says he has an unsafe swallow, and he has several previous admissions for aspiration
pneumonia. As such, he has been placed absolutely nil by mouth. His observations are stable, he is apyrexial and
mucus membranes are moist. He is not in any pain.

He has no other medical history and appears well.

He is admitted to the ward. What is the single most important medication to prescribe when admitting him?
A)Co-careldopa
B)Ramipril
C) Morphine
D) Dopamine agonist patch
E) Paracetamol
QUESTION 4
• A 68-year-old man presents to his GP with a one-year history of progressive 'slowing'. He describes a general loss
of dexterity, smaller handwriting and his wife has described his face as being 'less expressive'. He has noticed a
subtle tremor in the left hand over the past few months, which is more pronounced when sat listening to the radio.

On examination, he has mask-like facies, mild left-sided bradykinesia and cogwheel rigidity.

Given the likely diagnosis, which finding points towards idiopathic disease rather than a drug-induced disease?
A)Age of onset
B)Asymmetrical symptoms
C)Bradykinesia
D)Cogwheel rigidity
E)Micrographia
QUESTION 5
• A 46-year-old male presented to the emergency department with a 3-week history of “tightness” and “stiffness” in his
upper and lower limbs. He reports that he feels as if he has 'pulled a muscle' in his left leg. He has a background
medical history of well-controlled type two diabetes mellitus, multiple sclerosis, hypertension and
hypercholesterolaemia.

On examination, he has brisk deep tendon reflexes and hypertonia.

Given the likely diagnosis, what is the first-line treatment?


• A)Baclofen
• C)Botulinum toxin injection
• D)Diazepam
• E)Physiotherapy
• F)Pregabalin
QUESTION 6
• A 38-year-old female presents to her general practitioner with a 2-week history of urinary
frequency and incontinence. She is normally well but suffered from painful eye movements
and visual disturbance 6 months ago which have since largely resolved. A urine dipstick is
performed, which returns no abnormal findings.

Which of the following imaging modalities would best identify this patient's likely diagnosis?
• A) CT with contrast
• B) CT without contrast
• C) MRI with contrast
• D) MRI without contrast
QUESTION 7
• A 65-year-old man presents to neurology outpatients after being referred by his GP for muscle weakness.

Over the last few months, he has experienced weakness and clumsiness, such as dropping items and tripping while walking. He has also noticed he is
coughing when eating. His symptoms have been gradually worsening and do not vary significantly throughout the day.

He is finding it difficult to continue with his daily activities and is requiring assistance from his wife, who commented that his arms and legs look thinner
than usual.

What features on examination would support the most likely diagnosis?

• A)Dysdiadochokinesia and ataxia


• B)Extraocular muscle weakness on repeated eye movement
• C) Fasciculations and hypertonia
• D) Reduced proprioception
• E) Sensory loss and paraesthesia
QUESTION 8
• A 61-year-old man presents to his GP with recurrent falls over the past few months. He reports that his legs feel weak
and he has recently had difficulty doing up the buttons on his shirt. He reports no other symptoms and is otherwise
well. His past medical history includes hypertension for which he takes amlodipine. On examination, he
demonstrates bilateral foot drop with a high stepping gait. Power is reduced throughout both upper and lower limbs.
Reflexes were brisk in the upper limbs but the knee and ankle reflexes could not be elicited. He demonstrated
upgoing plantars. Coordination and sensation were intact throughout.

What is the most likely diagnosis?


A)Guillain-Barre syndrome
B)Amyotrophic lateral sclerosis
C)Cervical spondylotic myelopathy
D)Charcot-Marie-Tooth disease
E)Lambert-Eaton myasthenic syndrome
QUESTION 9
• A 70-year-old man presents to the Emergency Department (ED) with a 2-hour history of left arm and face weakness,
slurred speech and confusion that has since resolved.

He has a past medical history of hypertension, benign prostate hyperplasia, type 2 diabetes and ischaemic heart disease.

The patient has his observations performed and a collateral history is taken from his wife.

What is the most appropriate next step in ED?


• A)Blood alcohol level measurement
• B)Blood glucose measurement
• C)CT head
• D)Electroencephalogram (EEG)
• E)MRI head
QUESTION 10
• A 74-year-old man attends the emergency department with his wife complaining of weakness in his right
arm and leg, reduction in his vision, and dysphasia. His wife tells you that this started around half an hour
ago and she phoned an ambulance immediately. The patient is sent for a CT head and this excluded
haemorrhagic stroke. He was subsequently sent for a CT contrast angiography and this showed confirmed
occlusion of the proximal anterior circulation.

How should this patient be managed in the acute setting?


• A)Alteplase plus thrombectomy
• B)Alteplase alone
• C)Thrombectomy alone
• D)Aspirin alone
• E)Aspirin and clopidogrel
QUESTION 11
• A 70-year-old man presents with falls and weakness. He is unsure when it started, but says it has been over the last
day and fell after standing up from sitting. He has a history of recurrent falls, anxiety, and type 2 diabetes and he
takes metformin and bisoprolol. On examination, his speech is non-fluent and difficult, and his repetition is impaired.
His right face, arm, and leg are weak, and there is a loss in the right half of his visual field bilaterally. His blood
pressure is 96/75 mmHg, capillary blood glucose is normal, his oxygen saturations are 95%, and a CT scan is
unremarkable.

What is the most likely diagnosis?


• A)Chronic subdural haematoma
• B)Left-sided partial anterior circulation infarct
• C)Left-sided total anterior circulation infarct
• D)Orthostatic hypotension secondary to beta-blocker
• E)Right-sided total anterior circulation infarct
QUESTION 12
• A 67-year-old male presents with severe lower back pain that radiates down one of his legs. There
is no history of injury though he does a manual job. On examination, he has reduced perianal
sensation and reduced anal tone.

With regards to this patient's diagnosis, which of the following would represent a late sign and
indicate potentially irreversible damage?
• A)Positive sciatic stretch test
• B)Reduced perianal sensation
• C)Tingling of his right leg
• D)Urinary incontinence
• E)Reduced anal tone

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