Mock Neurology Module 5

A 35-year-old woman presents with recurrent transient ischaemic attacks characterised by right hemiparesis and expressive aphasia.

CT angiography shows bilateral stenosis of the distal internal carotid arteries and proximal middle cerebral arteries, with a characteristic “puff of smoke” network of small collateral vessels.

Which of the following is the most appropriate next investigation to confirm the diagnosis and assess operability?

Which of the following is considered a negative symptom in myopathy?

Which of the following is the most characteristic early clinical feature of Emery-Dreifuss muscular dystrophy?

In anti-synthetase syndrome, which of the following antibodies is least associated with interstitial lung disease?

A 45-year-old woman presents with a three-month history of progressive proximal muscle weakness, with difficulty rising from a chair and climbing stairs. Creatine kinase (CK) is 4000 U/L (reference <170 U/L female). Other serologies are pending.

Which condition is most likely responsible?

Which muscle disorder is correctly matched with its causative gene?

Which myopathy is most strongly associated with anti-NT5C1A (cytosolic 5′-nucleotidase 1A) antibodies?

Which of the following drugs/substances is NOT known to cause rhabdomyolysis with myoglobinuria?

Which best describes the main pathophysiology of myasthenia gravis?

Which of the following is the most common presenting symptom of myasthenia gravis (MG)?

A 45-year-old woman has three months of progressive fatigable facial and proximal limb weakness.

Which test is the most sensitive for confirming a neuromuscular-transmission defect?

A 45-year-old woman with anti-MuSK myasthenia gravis has relapsing bulbar-predominant weakness despite prednisone 80 mg/day and azathioprine 200 mg/day, with only transient benefit from intravenous immunoglobulin.

What is the most appropriate next step?

Which is NOT a commonly reported neurological autoimmune complication of immune checkpoint inhibitor therapy?

Which is a clear indication for thymectomy in myasthenia gravis?

A 35-year-old man has one week of progressive symmetric limb weakness with areflexia and normal sensation.

Nerve conduction studies show reduced CMAPs with preserved SNAPs.

CSF shows albuminocytological dissociation.

What is the most likely diagnosis?

A 45-year-old woman has five days of diplopia, dysphagia, ataxia and daytime somnolence.

Examination reveals ophthalmoplegia, facial weakness, ataxia, hyporeflexia and impaired consciousness.

Investigations:

• MRI shows T2-hyperintense brainstem lesions
• CSF shows albuminocytological dissociation
• Anti-GQ1b antibodies are positive.

What is the most likely diagnosis?

A 58-year-old man has progressive limb weakness, fasciculations and dysarthria, with combined upper and lower motor neuron signs, intact sensation, and EMG showing denervation and reinnervation.

Which criterion supports amyotrophic lateral sclerosis?

In motor neuron disease, “split-hand syndrome” refers to:

Which motor neuron disease phenotype is typically associated with the worst prognosis for survival?

A 32-year-old woman presents with double vision for three days.

On examination, when she looks to the right, her left eye fails to adduct fully and her right eye shows abducting nystagmus. Convergence is preserved. Visual acuity is normal.

She reports an episode of transient left leg weakness that resolved spontaneously 18 months ago.

MRI brain demonstrates a T2 hyperintense lesion in the dorsal pons.

Where is the lesion responsible for her ocular findings?

A 58-year-old man with hypertension presents with sudden-onset vertigo, vomiting, hoarseness and difficulty swallowing.

On examination he has a right-sided partial ptosis and miosis, loss of pain and temperature sensation on the right side of the face and the left side of the body, and gait ataxia veering to the right. There is no limb weakness.

MRI shows a wedge-shaped infarct.

Where is the lesion?

A 45-year-old man presents with ascending symmetrical limb weakness over five days, following a diarrhoeal illness two weeks earlier.

On examination he has flaccid weakness (power 3/5 lower limbs, 4/5 upper limbs), globally absent reflexes and mild distal sensory loss.

There is no sensory level. CSF shows protein 1.2 g/L (reference 0.15–0.45 g/L) with 2 cells/µL. Nerve conduction studies show prolonged distal motor latencies and conduction block.

Where is the primary site of the pathology?

A 28-year-old woman presents after a stab wound to the back.

On examination she has weakness of the right leg (power 2/5) with a right-sided extensor plantar response, loss of vibration and proprioception in the right leg, and loss of pain and temperature sensation in the left leg below the umbilicus. Light touch is relatively preserved.

Where is the lesion?

A 54-year-old man presents with a two-week history of right foot drop noticed after a prolonged period of squatting while laying tiles. He reports numbness over the dorsum of the right foot but no back pain.

On examination there is weakness of right ankle dorsiflexion (power 2/5) and foot eversion (2/5), but ankle inversion (4+/5) and plantar flexion are normal.

Sensation is reduced over the dorsum of the foot and the lateral aspect of the lower leg.

The ankle jerk is preserved and there is a palpable, tender thickening at the neck of the right fibula. Straight leg raise is negative.

Nerve conduction studies show conduction block of the common peroneal nerve across the fibular head with normal tibial and sural responses.

Where is the lesion?

A 62-year-old woman presents with fluctuating drooping of both eyelids and difficulty chewing that worsens towards the end of the day. She also reports intermittent double vision.

On examination, reflexes and sensation are normal, and there is fatigable ptosis.

Repetitive nerve stimulation shows a decremental response. Anti-acetylcholine receptor antibodies are positive. A CT chest is arranged.

Where is the site of the pathology?

A 40-year-old man presents with acute lower back pain radiating down the lateral aspect of the right leg to the dorsum of the foot after lifting a heavy object.

On examination he has weakness of the right great toe dorsiflexion and foot eversion, and reduced sensation over the dorsum of the foot and lateral shin. The ankle jerk is preserved. Straight leg raise reproduces his leg pain at 40 degrees.

Where is the lesion?

A 66-year-old man with poorly controlled hypertension presents with sudden severe headache and double vision. On examination he has complete ptosis of the right eye. The right eye is deviated “down and out”, and the right pupil is fixed and dilated at 6 mm. The left pupil is 3 mm and reactive. There is no limb weakness.

What is the most appropriate next step in management?

A 52-year-old woman with type 2 diabetes and hypothyroidism presents with numbness and tingling of the right thumb, index and middle fingers, worse at night.

On examination she has reduced sensation over the palmar aspect of the first three digits, wasting of the thenar eminence and weakness of thumb abduction.

Sensation over the thenar eminence itself is preserved.

Nerve conduction studies show prolonged distal median motor and sensory latencies across the wrist.

Where is the lesion?

A 70-year-old man presents with visual disturbance.

On confrontation testing he has a left homonymous hemianopia with macular sparing. Visual acuity is 6/6 bilaterally. There is no dysphasia, neglect, or limb weakness. Pupillary responses are normal. MRI is performed.

Where is the lesion?

A 58-year-old man presents with 8 months of progressive weakness.

On examination he has wasting and fasciculations of the tongue and both hands, brisk reflexes throughout including a positive Hoffmann sign and extensor plantar responses, and spastic dysarthria.

Sensation is entirely normal and there is no bladder disturbance.

Nerve conduction studies are normal; EMG shows widespread fibrillations and fasciculations with chronic reinnervation across multiple regions.

Where is the site of the pathology?