Neurology Prometric exam questions

The Neurology exam in every GCC country

From each regulator's own exam pages; anything a regulator does not publish says so. Assessment mode also depends on your licence title, so confirm your route on your eligibility notice.

12 worked Neurology practice questions

Original exam-style questions from the bank, spread across the blueprint domains. They are not real or recalled exam questions. Try each one, then open the answer for the rationale and reference. None of them appear in the free mock exam, so reading them will not spoil it.

1. Question 1 · Cerebrovascular Disorders

   A 42-year-old woman with a history of migraine with aura and two prior lacunar strokes has a strong family history of early stroke and dementia. MRI shows confluent white matter hyperintensities involving the anterior temporal poles and external capsules. Which diagnosis and confirmatory test are most appropriate?

   1. ACADASIL; NOTCH3 gene testing
   2. BFabry disease; alpha-galactosidase A activity in a female patient as the definitive test
   3. CMultiple sclerosis; CSF oligoclonal bands
   4. DMELAS; serum lactate
   Show answer and explanation

   Correct answer: A. CADASIL; NOTCH3 gene testing

   Anterior temporal pole and external capsule white matter hyperintensities in a young adult with migraine with aura, recurrent lacunar strokes and a dominant family history are highly characteristic of CADASIL, confirmed by NOTCH3 mutation analysis (skin biopsy showing granular osmiophilic material is an alternative). Enzyme activity is unreliable in heterozygous females with Fabry disease, MS lesions are periventricular and ovoid with Dawson fingers rather than temporopolar, and MELAS produces stroke-like lesions that cross vascular territories with cortical involvement.

   Reference: Chabriat et al., Lancet Neurology 2009; Bradley's Neurology in Clinical Practice, 8th ed. (2022)

2. Question 2 · Cephalgia and Headache Disorders

   A 38-year-old woman has 14 attacks per day of severe right orbital pain, each lasting about 15 minutes, accompanied by right lacrimation, conjunctival injection and nasal congestion. MRI of the brain including pituitary views is normal. Which management step is both diagnostic and therapeutic?

   1. AA trial of high-flow oxygen at 12 L/min for 15 minutes
   2. BA supervised trial of indometacin titrated to an adequate dose
   3. CA trial of carbamazepine 200 mg twice daily
   4. DA trial of subcutaneous sumatriptan 6 mg for each attack
   Show answer and explanation

   Correct answer: B. A supervised trial of indometacin titrated to an adequate dose

   Multiple short attacks (2-30 minutes) occurring many times daily with prominent cranial autonomic features is the pattern of paroxysmal hemicrania, which by definition shows an absolute response to adequate doses of indometacin; the indometacin trial therefore confirms the diagnosis and treats the condition (with gastroprotection). Oxygen and subcutaneous sumatriptan abort cluster attacks, which are longer (15-180 minutes) and far less frequent, and carbamazepine is used for trigeminal neuralgia, where autonomic features are absent or minimal.

   Reference: ICHD-3, International Headache Society 2018; Continuum (AAN) Headache issue, 2021

3. Question 3 · Neuromuscular Disorders

   A 26-year-old woman reports stiffness of the hands and eyelids that is mild on the first contraction and becomes progressively worse with each repeated movement, and is markedly aggravated by cold weather. Her father is similarly affected. Which statement best characterises this disorder?

   1. AIt is myotonia congenita due to CLCN1 mutations, in which myotonia improves with repeated activity (the warm-up phenomenon)
   2. BIt is neuromyotonia due to voltage-gated potassium channel complex antibodies, in which stiffness persists during sleep
   3. CIt is myotonic dystrophy type 1, in which stiffness is accompanied by distal weakness, cataracts and cardiac conduction disease
   4. DIt is paramyotonia congenita due to SCN4A mutations, in which myotonia paradoxically worsens with repeated activity and with cold
   Show answer and explanation

   Correct answer: D. It is paramyotonia congenita due to SCN4A mutations, in which myotonia paradoxically worsens with repeated activity and with cold

   Myotonia that intensifies with repetition (paradoxical myotonia) and is provoked by cold, inherited in an autosomal dominant pattern, defines paramyotonia congenita caused by gain-of-function mutations in the skeletal muscle sodium channel gene SCN4A; mexiletine is the treatment of choice. Chloride channel myotonia congenita shows the opposite warm-up phenomenon; myotonic dystrophy has multisystem features absent here; acquired neuromyotonia is immune-mediated with continuous muscle fibre activity and autonomic features rather than cold-induced paradoxical myotonia.

   Reference: Statland JM and Barohn RJ, Continuum: Lifelong Learning in Neurology (2013), Muscle channelopathies; GeneReviews, Non-dystrophic Myotonia (updated 2021)

4. Question 4 · Neuro-Infectious Disorders

   A 19-year-old man with untreated sinusitis presents with high fever, severe headache, rapidly declining consciousness, seizures and a dense hemiparesis over 24 hours. MRI shows a crescentic extra-axial collection over the convexity with restricted diffusion, marked dural enhancement and midline shift. What is the most appropriate immediate management?

   1. AIntravenous antibiotics alone, with surgery only if there is no improvement after 72 hours
   2. BMannitol and antiseizure medication alone
   3. CAnticoagulation for presumed septic venous thrombosis before any surgery
   4. DEmergency neurosurgical drainage (craniotomy or burr holes) together with intravenous antibiotics and antiseizure medication
   Show answer and explanation

   Correct answer: D. Emergency neurosurgical drainage (craniotomy or burr holes) together with intravenous antibiotics and antiseizure medication

   Subdural empyema is a neurosurgical emergency: the collection is in a space with no barrier to spread, deterioration is rapid, and delay in drainage is associated with markedly worse outcome, so urgent evacuation plus antibiotics and seizure control is required. Medical therapy alone or osmotherapy alone does not evacuate the pus, and anticoagulation for possible septic venous thrombosis is a secondary consideration that must not delay operative drainage.

   Reference: Bradley's Neurology in Clinical Practice, 8th edition (2022); Brouwer & van de Beek, Epidemiology, diagnosis and treatment of intracranial suppuration (2017)

5. Question 5 · Cognitive Disorders

   A 28-year-old man of consanguineous parents has progressive cognitive decline and a spastic ataxic gait. He had bilateral cataracts removed at age 9, has chronic diarrhoea since childhood, and has firm nodular swellings over both Achilles tendons. MRI shows dentate nucleus hyperintensity and cerebellar white matter change. Which is the most likely diagnosis?

   1. ANiemann-Pick disease type C
   2. BCerebrotendinous xanthomatosis
   3. CX-linked adrenoleukodystrophy
   4. DWilson disease
   Show answer and explanation

   Correct answer: B. Cerebrotendinous xanthomatosis

   Cerebrotendinous xanthomatosis is an autosomal recessive CYP27A1 defect and the combination of juvenile cataracts, chronic infantile diarrhoea, tendon xanthomas, dementia and spastic ataxia with dentate nucleus signal change is nearly pathognomonic; the diagnosis is confirmed by elevated plasma cholestanol and is treatable with chenodeoxycholic acid, so early recognition matters greatly. Niemann-Pick type C causes vertical supranuclear gaze palsy, splenomegaly and gelastic cataplexy without tendon xanthomas. Adrenoleukodystrophy is X-linked with adrenal insufficiency and posterior parieto-occipital white matter change, and Wilson disease produces Kayser-Fleischer rings and hepatic dysfunction rather than tendon xanthomas or cataracts.

   Reference: Nie S et al., Cerebrotendinous xanthomatosis: a comprehensive review, Orphanet J Rare Dis 2014; Bradley's Neurology in Clinical Practice, 8th edition (2022)

6. Question 6 · Neurology of Systemic Diseases (including Nutritional, Metabolic and Toxic)

   A 52-year-old woman who had a gastric bypass eight years ago presents with a spastic paraparesis, impaired vibration and joint position sense, a sensory ataxia and a macrocytic anaemia with neutropenia. Serum vitamin B12, methylmalonic acid and homocysteine are all normal. She has been taking a high-dose zinc supplement for her skin. What is the most likely deficiency?

   1. ACopper
   2. BFolate
   3. CVitamin E
   4. DNiacin
   Show answer and explanation

   Correct answer: A. Copper

   Acquired copper deficiency, typical after bariatric surgery or with excess zinc intake (zinc induces enterocyte metallothionein, which sequesters copper), produces a myeloneuropathy clinically indistinguishable from subacute combined degeneration but with normal B12, methylmalonic acid and homocysteine, plus anaemia and neutropenia. Folate deficiency causes macrocytosis with raised homocysteine and rarely a myelopathy. Vitamin E deficiency causes spinocerebellar ataxia with retinopathy but not neutropenia, and niacin deficiency causes pellagra with dermatitis, diarrhoea and dementia.

   Reference: Kumar, Copper deficiency myelopathy (human swayback) (Mayo Clinic Proceedings, 2006); AAN Continuum: Neurologic Complications of Systemic Disease

7. Question 7 · Professionalism, Ethics and Communication

   The same patient, alone with you the following morning, looks at you directly and asks: 'Doctor, is this a brain cancer, and how long do I have?' Her sons have asked you not to tell her. What should you do?

   1. AReply that you cannot discuss this without her sons present
   2. BSay that the tests are inconclusive and that more time is needed
   3. CTell her the family has asked you not to discuss the results
   4. DAnswer her question honestly and sensitively at the level of detail she is asking for, check her understanding, and afterwards explain to the sons why you were obliged to respond
   Show answer and explanation

   Correct answer: D. Answer her question honestly and sensitively at the level of detail she is asking for, check her understanding, and afterwards explain to the sons why you were obliged to respond

   When a competent patient directly asks for her diagnosis, the duty of veracity and her right to information override a family's request for concealment; deceiving her would be a clear breach of professional integrity. Deflection or an outright lie ('inconclusive') damages trust irreparably once the truth emerges. Telling her only that the family has forbidden disclosure evades her question and sets her against her sons; the correct sequence is to answer her, then talk the family through the reasoning and support them.

   Reference: WMA International Code of Medical Ethics (revised 2022); GMC Good Medical Practice (2024)

8. Question 8 · Epilepsy and Paroxysmal Disorders

   A 14-year-old boy with Lennox-Gastaut syndrome has multiple daily atonic drop attacks causing repeated facial injuries despite four antiseizure medications and the ketogenic diet. MRI is normal and EEG shows diffuse slow spike-and-wave with no single focus. Which surgical option is most appropriate?

   1. ASelective amygdalohippocampectomy
   2. BFunctional hemispherotomy
   3. CMultiple subpial transections of the motor cortex
   4. DCorpus callosotomy
   Show answer and explanation

   Correct answer: D. Corpus callosotomy

   Corpus callosotomy is an established palliative procedure that interrupts interhemispheric spread and is most effective specifically for atonic drop attacks in generalised epileptic encephalopathies without a resectable focus, markedly reducing injury even though it rarely produces seizure freedom. Amygdalohippocampectomy requires a mesial temporal focus and hemispherotomy requires diffuse unilateral hemispheric pathology, neither of which is present with normal MRI and generalised discharges. Multiple subpial transection is used for epileptogenic tissue within eloquent cortex, not for generalised drop attacks.

   Reference: Cross JH et al., Proposed criteria for referral and evaluation of children for epilepsy surgery, Epilepsia 2006; Bradley's Neurology in Clinical Practice, 8th edition, 2022

9. Question 9 · Neuro-oncology, Neuro-ophthalmology, Neuro-otology and Sleep Neurology

   A 61-year-old diabetic man has 12 hours of continuous vertigo, vomiting and unsteadiness. He has spontaneous left-beating horizontal nystagmus that does not change direction on gaze, a normal head impulse test bilaterally, and a vertical misalignment on cover testing. What do these HINTS findings indicate?

   1. AA peripheral cause, most likely vestibular neuritis
   2. BBenign paroxysmal positional vertigo of the posterior canal
   3. CA central cause, most likely posterior circulation stroke
   4. DMeniere disease
   Show answer and explanation

   Correct answer: C. A central cause, most likely posterior circulation stroke

   In acute vestibular syndrome a normal (negative) head impulse test, direction-changing nystagmus, or skew deviation on cover testing each point to a central lesion, and HINTS performed by a trained examiner is more sensitive than early MRI for posterior circulation stroke. Vestibular neuritis gives an abnormal head impulse with a corrective saccade, unidirectional nystagmus and no skew. BPPV causes brief positional attacks rather than continuous vertigo, and Meniere disease produces episodic vertigo with fluctuating hearing loss and aural fullness.

   Reference: Kattah JC et al., HINTS to diagnose stroke in the acute vestibular syndrome, Stroke 2009; Barany Society diagnostic criteria

10. Question 10 · Neuro-Immunological and Demyelinating Disorders

    A 40-year-old woman with biopsy-proven neurosarcoidosis causing a progressive hypothalamic-pituitary syndrome and parenchymal brainstem lesions continues to deteriorate clinically and radiologically despite nine months of high-dose corticosteroids and an adequate trial of methotrexate. Which is the most appropriate next treatment?

    1. AInfliximab
    2. BNatalizumab
    3. CMonthly intravenous immunoglobulin
    4. DPlasma exchange
    Show answer and explanation

    Correct answer: A. Infliximab

    TNF-alpha is central to granuloma formation, and infliximab has the best evidence of any third-line agent for refractory neurosarcoidosis, producing radiological and clinical improvement in a majority of steroid- and antimetabolite-refractory patients; screening for latent tuberculosis and hepatitis B is required first. Natalizumab, IVIG and plasma exchange have no established role in granulomatous CNS disease.

    Reference: Stern BJ et al. JAMA Neurology 2018; Gelfand JM et al. Infliximab for the treatment of CNS sarcoidosis: a multi-institutional series. Neurology 2017

11. Question 11 · Movement Disorders

    A 58-year-old woman with Parkinson disease reports that her morning dose of levodopa often takes more than 90 minutes to take effect and that the dose taken with her large midday meat-based meal frequently produces no benefit at all. She takes all doses with food and complains of bloating and constipation. Which is the most appropriate initial management?

    1. AIncrease every levodopa dose by 50 per cent
    2. BAdd amantadine
    3. CAdd a dopamine agonist at midday
    4. DGive levodopa 30 to 60 minutes before meals, redistribute dietary protein away from daytime doses, and treat the constipation and delayed gastric emptying
    Show answer and explanation

    Correct answer: D. Give levodopa 30 to 60 minutes before meals, redistribute dietary protein away from daytime doses, and treat the constipation and delayed gastric emptying

    Delayed-on and dose failure in Parkinson disease usually reflect delayed gastric emptying and competition between dietary large neutral amino acids and levodopa for intestinal and blood-brain barrier transport, so timing, protein redistribution and treatment of gastroparesis and constipation are the first interventions. Simply increasing the dose risks peak-dose dyskinesia without correcting absorption; amantadine treats dyskinesia rather than absorption failure; adding an agonist adds adverse effects without addressing the mechanism.

    Reference: Fox SH et al. MDS evidence-based medicine review update on treatments for the motor symptoms of Parkinson's disease. Movement Disorders, 2018; Bradley and Daroff's Neurology in Clinical Practice, 8th edition, 2022

12. Question 12 · Cognitive Disorders

    The family of a man with dementia and persistent, distressing persecutory delusions ask about the risks of the antipsychotic you have proposed. Which statement is the most accurate counselling point?

    1. AAtypical antipsychotics reduce all-cause mortality in dementia by decreasing agitation-related injury
    2. BThe increased cerebrovascular risk applies to Alzheimer disease but not to vascular or mixed dementia
    3. CAntipsychotics are associated with an increased risk of death and cerebrovascular events in dementia, so they should be used at the lowest effective dose for the shortest time, with a documented indication and regular review for withdrawal
    4. DOnce an antipsychotic is effective it should be continued indefinitely, as withdrawal reliably causes relapse
    Show answer and explanation

    Correct answer: C. Antipsychotics are associated with an increased risk of death and cerebrovascular events in dementia, so they should be used at the lowest effective dose for the shortest time, with a documented indication and regular review for withdrawal

    Meta-analyses and regulatory reviews demonstrate roughly a 1.5 to 1.7-fold increase in mortality and an increased incidence of stroke with both typical and atypical antipsychotics in elderly patients with dementia, which underpins the boxed warning and the guidance to prescribe the lowest effective dose for the shortest period with regular review. The risk is not confined to any single dementia subtype and is at least as high in vascular and mixed disease. Withdrawal trials show that most patients can discontinue without relapse, so indefinite continuation is not justified, although patients with severe psychosis who initially responded strongly need particularly careful, gradual withdrawal.

    Reference: Schneider LS, Dagerman KS, Insel P, Risk of death with atypical antipsychotic drug treatment for dementia, JAMA 2005; NICE NG97 (2018); US FDA boxed warning on antipsychotics in elderly patients with dementia-related psychosis

Neurology reference books and guidelines

Every question in this bank names the textbook or guideline its answer rests on. These are the sources our Neurology questions cite most often - the reading list to revise from alongside the bank.

Where your regulator publishes a reading list, use the editions it names. Exam questions are not taken from any one book.