Mock Neurology Module 2

A 65-year-old man presents acutely to the emergency department with symptoms consistent with a transient ischaemic attack (TIA) and a low NIHSS score.

His symptoms resolved within an hour of presentation. Imaging reveals minor ischaemic changes with no large infarcts.

His past medical history includes atrial fibrillation, for which he is unable to tolerate oral anticoagulants.

Which of the following is the most appropriate management for this patient?

A 25-year-old woman presents to the clinic following a second episode of visual loss in her right eye accompanied by eye pain that worsens with movement.

The first episode occurred last month at her office and resolved spontaneously after several hours. Her medical history is unremarkable, and she has no known autoimmune disorders.

On examination, MRI shows inflammation of the optic nerve. Her cerebrospinal fluid shows normal glucose and protein with no oligoclonal bands. She is MOG-antibody positive and aquaporin-4 antibody negative. Serum ACE is negative, and her autoimmune screen (ANA/ENA) is negative.

Which of the following conditions is most likely associated with her clinical presentation?

Which of the following medications is least appropriate as the initial treatment for severe migraine?

A 15-year-old previously well male presents to the emergency department with sudden onset of flaccid paraplegia and urinary retention immediately after performing a backflip during gymnastics practice.

He describes a brief, sharp mid-thoracic back pain at onset and denies head or spine trauma.

Neurological examination reveals 0/5 power in both lower limbs, loss of pain and temperature sensation below T8, preserved proprioception and vibration sense, normal upper limb function, and no spinal tenderness.

MRI of the spine shows T2 hyperintensity and cord swelling at T6–T9 involving predominantly the anterior cord, without mass lesion or disc herniation.

CSF analysis is normal with no pleocytosis and mildly elevated protein.

Full blood count, electrolytes, and CRP are within normal limits.

Which of the following is the most likely diagnosis?

A 65-year-old woman with hypertension, hyperlipidaemia, and type 2 diabetes presents to the emergency department with acute ischaemic stroke, including left-sided weakness and slurred speech, within 3 hours of onset.

Her blood pressure is 180/100 mmHg and her blood glucose is 13.9 mmol/L (reference 4.0–7.8 mmol/L fasting; random <11.1 mmol/L).

Imaging reveals a distal middle cerebral artery occlusion beyond the dominant M2 segment (a medium-vessel occlusion).

Her NIHSS is 12. She takes aspirin, metformin, and atorvastatin, with no known allergies.

Which of the following is the most appropriate treatment recommendation?

An 84-year-old man presents for evaluation of a 5-year history of gradually worsening gait, a 2-year history of cognitive impairment, and urinary incontinence.

Twelve years ago he sustained a closed head injury causing a mild traumatic subarachnoid haemorrhage with loss of consciousness for five hours.

His current medications include zopiclone and multivitamins.

On examination he is afebrile with stable observations and a BMI of 27. His gait is slow, unsteady, and marked by small, shuffling, magnetic steps. Alertness, speech, posture, arm swing, and muscle tone are normal, and there is no tremor.

He scores 24/30 on the Mini-Mental State Examination, losing one point for orientation, three for serial calculation, and two for recall.

Full blood count, metabolic panel, serum vitamin B12, thyroid function, and urinalysis are normal.

What is the most likely diagnosis?

A 54-year-old man is assessed on the ward for leg weakness. He is an ex-smoker and drinks 15 standard drinks per week. His wife reports he is confused.

On examination, his MMSE is 20/30, he has an ataxic gait, bilateral pyramidal weakness, and impaired coordination.

Routine blood tests are normal. MRI of the head shows diffuse white matter changes, more prominent in the cerebellum than the cerebrum.

Which test would be most helpful in confirming the diagnosis?

A 60-year-old man of Māori descent presents with nystagmus on looking to the left, ataxia, and vertigo. He has a left-sided Horner’s syndrome. There is loss of pain and temperature sensation on the left side of the face and loss of pain and temperature sensation on the right side of the trunk and limbs.

Which of the following is the diagnosis?

Which of the following features is most suggestive of a cluster headache compared with a migraine attack?

Lumbar puncture should be preceded by CT of the head in all of the following patients suspected of having meningitis EXCEPT those with:

A 27-year-old woman is referred from the ophthalmology clinic with bilateral papilloedema. She is obese (BMI 35) with a history of renal calculi. She reports blurred vision for several months and a chronic dull left-sided headache.

On visual testing, the left eye showed an enlarged blind spot and both optic discs were swollen. Other cranial nerve and motor examination was unremarkable.

CT of the head was normal. Lumbar puncture demonstrated an opening pressure of 31 cmH₂O (reference <25 cmH₂O) with normal CSF glucose, protein, and cell count.

MRI and MR venography showed some narrowing of the dural venous sinuses but no thrombosis. The pituitary and remainder of the study were unremarkable.

Which of the following interventions would be least helpful?

Which of the following genetic abnormalities is most commonly implicated in familial amyotrophic lateral sclerosis (FALS)?

Which investigation is most useful in supporting the diagnosis of motor neuron disease?

A 30-year-old man presents with weakness of the tongue and mouth muscles and progressive limb weakness. He and his partner have been unable to conceive.

On examination there is muscle wasting, fasciculations (including perioral and tongue fasciculations), and gynaecomastia.

What is the most likely diagnosis?

In motor neuron disease, which of the following interventions is most likely to prolong survival?

What is the mechanism of action of Riluzole?

A patient who presents purely of lower motor neuron signs will likely to have?

A patient presenting purely with lower motor neuron signs and no systemic or endocrine features is most likely to have:

A 64-year-old man is reviewed in the multidisciplinary motor neuron disease clinic six months after diagnosis.

He and his family ask about factors that influence his prognosis.

Which of the following features is associated with a more favourable, rather than a worse, survival outcome in motor neuron disease?

What is the most common cause of death in motor neuron disease?

A 34-year-old woman with focal epilepsy, well controlled on carbamazepine 400 mg twice daily for the past three years, presents to the emergency department with a three-day history of unsteadiness, double vision, drowsiness, and nausea. She has had no seizures.

Ten days ago her general practitioner commenced a course of an antibiotic for a respiratory tract infection.

On examination she is drowsy but rousable, with horizontal gaze-evoked nystagmus, dysarthria, and a broad-based ataxic gait.

Her serum sodium is 126 mmol/L (reference 135–145 mmol/L) and her carbamazepine level is markedly elevated at 68 micromol/L (therapeutic reference 17–51 micromol/L).

She has taken no additional doses of her usual medication.

Which of the following recently prescribed medications is the most likely cause of her presentation?

A 58-year-old woman is referred to the endocrinology clinic after sustaining a low-trauma fracture of the distal radius following a fall from standing height. She has a 25-year history of focal epilepsy that has been well controlled on long-term monotherapy. She is postmenopausal, does not smoke, and has no family history of osteoporosis.

Investigations reveal a 25-hydroxyvitamin D level of 32 nmol/L (reference >50 nmol/L), a mildly elevated alkaline phosphatase, a low-normal serum calcium, and a raised parathyroid hormone.

Dual-energy X-ray absorptiometry demonstrates a lumbar spine T-score of –2.8 and a femoral neck T-score of –2.6.

Her treating team suspects that her long-term antiseizure medication has contributed to her bone disease.

Which of the following antiseizure medications is most strongly associated with this presentation?

A 24-year-old woman with genetic generalised epilepsy attends the neurology clinic for preconception counselling, as she and her partner are planning a pregnancy.

Her seizures, which include myoclonic jerks and generalised tonic-clonic seizures, have been fully controlled for the past two years. She is currently taking one antiseizure medication and is otherwise well.

She asks specifically which medications carry the greatest risk of harm to a developing fetus, as she is anxious to minimise this risk before conceiving.

Which of the following antiseizure medications is associated with the greatest teratogenic potential in pregnancy?

A 42-year-old man with drug-resistant focal epilepsy has been maintained on an antiseizure medication for the past 18 months after failing to achieve seizure control with several other agents.

At a routine clinic review he reports increasing difficulty seeing objects to the sides while walking and describes two recent episodes of bumping into door frames, although he has not noticed any blurring of central vision.

Formal automated perimetry demonstrates bilateral concentric constriction of the peripheral visual fields, and optical coherence tomography shows thinning of the retinal nerve fibre layer, while central visual acuity is preserved.

Which of the following antiseizure medications is the most likely cause of these findings?

Which of the following antiseizure medications is associated with weight loss?

A 22-year-old woman with epilepsy wishes to use a combined oral contraceptive pill.

Which of the following antiseizure medications would be most compatible with reliable contraception and carry the lowest teratogenic risk?

A 19-year-old woman is referred to the neurology clinic after a marked deterioration in her seizure control. Since her mid-teens she has experienced early-morning jerks of her upper limbs, often causing her to drop her cup at breakfast, and she has had two generalised tonic-clonic seizures, both on waking after late nights and alcohol.

She was recently diagnosed with epilepsy by another practitioner, who, presuming focal seizures, commenced an antiseizure medication. Since starting this drug her myoclonic jerks have become considerably more frequent, and she has had a further convulsive seizure.

Her electroencephalogram shows generalised 4–6 Hz polyspike-and-wave discharges with a photoparoxysmal response.

Which of the following medications is the most likely cause of the worsening of her seizures and should be avoided in this condition?

A 27-year-old woman with focal epilepsy is commenced on oral lamotrigine, which was rapidly uptitrated as an add-on therapy. She is already taking another antiseizure medication.

Two and a half weeks after starting lamotrigine she presents to the emergency department with a three-day history of fever, malaise, and a painful, rapidly progressive rash.

On examination, she has widespread dusky erythematous macules with early blistering and a positive Nikolsky sign, together with erosions of the oral and conjunctival mucosae, affecting approximately 8% of her body surface area.

Which of the following antiseizure medications most likely precipitated this significant cutaneous reaction? 

A 56-year-old man presents to the clinic with a several-year history of a bilateral, largely symmetrical tremor of both hands that is most apparent when he holds a cup, writes, or brings a spoon to his mouth.

The tremor is absent at rest and disappears during sleep. He has noticed that a glass of wine in the evening temporarily settles the tremor, and there is a strong family history, with his father and elder brother similarly affected.

On examination there is a postural and kinetic tremor of the upper limbs, a subtle head tremor, and no rigidity, bradykinesia, or other parkinsonian features. 

Which of the following treatments would be of NO benefit in this patient’s condition?

A 68-year-old man with newly diagnosed Parkinson disease is commenced on levodopa combined with a peripheral dopa-decarboxylase inhibitor for progressive bradykinesia, rigidity, and a resting tremor affecting his right hand.

Shortly after starting treatment, and again when his dose is increased two weeks later, he develops troublesome nausea and occasional vomiting, most noticeable in the period after taking each dose.

He has no confusion, no involuntary movements, and no gastrointestinal upset of any other kind. He is otherwise well and is keen to continue treatment given the benefit to his motor symptoms.

Which of the following is the most common early adverse effect of levodopa, particularly on initiation or dose increase?

A 72-year-old woman with a 9-year history of Parkinson disease is reviewed in the movement disorders clinic. Although her motor symptoms initially responded well to levodopa, she has developed increasingly troublesome involuntary writhing movements of her limbs and trunk that occur at the peak of each dose. These dyskinesias are now interfering with her daily activities and are a source of distress.

After discussion, her neurologist adds amantadine specifically to reduce these levodopa-induced dyskinesias.

Amantadine exerts its principal therapeutic effect in this setting by acting as an antagonist at which of the following receptors?

A 68-year-old patient with early Alzheimer’s disease enrolled in a trial of an amyloid-targeting monoclonal antibody presents after four months of treatment with sudden confusion, headache, and mild right-sided weakness.

MRI of the brain reveals bilateral asymmetric white matter hyperintensities suggestive of vasogenic oedema, predominantly in the occipital regions, without cerebral haemorrhage.

The clinical and imaging findings are most consistent with which diagnosis?

A 75-year-old patient with atrial fibrillation on warfarin presents with sudden right-sided weakness and aphasia. Imaging confirms intracerebral haemorrhage and the INR is significantly elevated (reference therapeutic range 2.0–3.0 for atrial fibrillation).

What is the most appropriate next step to improve survival and reduce the likelihood of discharge to hospice, given the importance of rapid treatment?

A 62-year-old man presents to a tertiary hospital in New Zealand 6 hours after the sudden onset of right-sided hemiplegia and global aphasia.

Non-contrast CT and CT angiography confirm occlusion of the proximal left middle cerebral artery (M1 segment), and CT perfusion demonstrates a large established ischaemic core.

He was last known well 6 hours ago, is not eligible for intravenous thrombolysis, and has a good pre-morbid functional status.

Which of the following is the most appropriate next step in management?

A 55-year-old woman with atrial fibrillation presents with acute left-sided weakness and dysarthria. CT angiography confirms occlusion of the right middle cerebral artery.

A non-contrast CT of the head is performed to assess the extent of early ischaemic change using the Alberta Stroke Program Early CT Score (ASPECTS), which is calculated as 4, indicating a large area of established infarction.

The stroke team discusses whether this low score should preclude her from mechanical thrombectomy.

Which of the following statements best describes the ASPECTS and its role in decision-making for reperfusion therapy?