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  • Question 1 - A 57-year-old architect presents with weakness of the right hand. You note global...

    Incorrect

    • A 57-year-old architect presents with weakness of the right hand. You note global wasting of the small hand muscles. There is also sensory loss over the medial border of the forearm around the elbow. Which of the following nerve roots is damaged?

      Your Answer: C6

      Correct Answer: T1

      Explanation:

      This patient has Klumpke’s paralysis due to damage to the T1 nerve root. This root eventually supplies the median and ulnar nerves. The ulnar nerve supplies all of the intrinsic hand muscles except for those of the thenar eminence and the first and second lumbricals, which are innervated by the median nerve.

    • This question is part of the following fields:

      • Neurology
      452.4
      Seconds
  • Question 2 - A 53 year-old dancer presents to the ED with increasing weakness. She has...

    Incorrect

    • A 53 year-old dancer presents to the ED with increasing weakness. She has no pertinent past medical history aside from a recent diarrhoeal illness, which she attributes to an undercooked chicken meal. Her husband says that she has been unable to get up out of a chair for the past day. Upon examination, there is bilateral limb weakness and areflexia noted, but it is more severe in the lower limbs. You notice that if she lies flat in the bed, her oxygen saturations fall by around 2% on the pulse oximeter and she is unable to perform spirometry. Which of the following represents the most appropriate immediate management of choice in this patient?

      Your Answer: Plasma exchange

      Correct Answer: ITU review for consideration of ventilation

      Explanation:

      This woman has a history that is suggestive of Guillain– Barré syndrome. This may be precipitated by Campylobacter, and her history of recent diarrhoeal illness is pointing towards that. Certain features point to a poor prognosis, including rapidity of onset, reduced vital capacity or respiratory failure, age >40 and reduced amplitude of compound muscle action potential. Her inability to perform spirometry and desaturating whilst lying flat are suggestive of impending respiratory muscle weakness. Review for consideration of ventilation is recommended. Further management of choice for Guillain-Barre syndrome is IV immunoglobulins. Steroids have no value in the treatment of the condition.

    • This question is part of the following fields:

      • Neurology
      29.6
      Seconds
  • Question 3 - Which of the following peripheral neuropathy inducing clinical diagnoses is predominantly associated with...

    Incorrect

    • Which of the following peripheral neuropathy inducing clinical diagnoses is predominantly associated with sensory loss?

      Your Answer: Hereditary sensorimotor neuropathies

      Correct Answer: Uraemia

      Explanation:

      Diseases that cause predominantly sensory loss include diabetes, uraemia, leprosy, alcoholism, vitamin B12 deficiency, and amyloidosis.
      Those that cause predominantly motor loss include Guillain-Barre syndrome, porphyria, lead poisoning, hereditary sensorimotor neuropathies, chronic inflammatory demyelinating polyneuropathy, and diphtheria.

    • This question is part of the following fields:

      • Neurology
      8.8
      Seconds
  • Question 4 - Which of the following types of motor neuron diseases carries the worst prognosis?...

    Correct

    • Which of the following types of motor neuron diseases carries the worst prognosis?

      Your Answer: Progressive bulbar palsy

      Explanation:

      Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognised including amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy. In some patients however, there is a combination of clinical patterns. In progressive bulbar palsy there is palsy of the tongue and muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei. This carries the worst prognosis.

    • This question is part of the following fields:

      • Neurology
      5.9
      Seconds
  • Question 5 - Which of the following may cause a downbeat nystagmus? ...

    Incorrect

    • Which of the following may cause a downbeat nystagmus?

      Your Answer: Unilateral medial longitudinal fasciculus lesion

      Correct Answer: Chiari type I malformation

      Explanation:

      Downbeat nystagmus (DBN) suggests a lesion in the lower part of the medulla. Chiari Type I malformation usually presents with symptoms due to brain stem and lower cranial nerve dysfunction, which includes DBN.

    • This question is part of the following fields:

      • Neurology
      144.2
      Seconds
  • Question 6 - A 29-year-old physiotherapist with a history of bilateral vitreous haemorrhage is referred due...

    Incorrect

    • A 29-year-old physiotherapist with a history of bilateral vitreous haemorrhage is referred due to progressive ataxia. Which of the following is the most likely diagnosis?

      Your Answer: Neurofibromatosis type II

      Correct Answer: Von Hippel-Lindau syndrome

      Explanation:

      Retinal and cerebellar haemangiomas are key features of Von Hippel-Lindau syndrome. Retinal haemangiomas are bilateral in 25% of patients and may lead to vitreous haemorrhage. Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3.

    • This question is part of the following fields:

      • Neurology
      31.6
      Seconds
  • Question 7 - A 43-year-old police officer is admitted with a history of unsteadiness and slurring...

    Incorrect

    • A 43-year-old police officer is admitted with a history of unsteadiness and slurring of speech. This has worsened over a period of three months. He complains of a tremor affecting his right hand and diplopia on right lateral gaze. He smokes 30 cigarettes a day and takes regular diclofenac for his arthritis. On examination, he is dysarthric and feels nauseated. Fundoscopy is normal, however there is marked horizontal nystagmus and evidence of a right VI nerve palsy. There also appears to be some mild facial weakness on the right side. Upon conducting Weber’s test, a louder tone is heard in the left ear. On conducting the Rinne test, both ears are normal. On examination of the upper limb, there is a right intention tremor and dysdiadochokinesis. Tone, power and reflexes are normal. On examination of the lower limb, tone, power and reflexes are normal, however he appears to walk with a broad-based gait and is leaning to the right. Lumbar puncture: Opening pressure 13 cm H20 (5–18) Protein 0.67 g/l (0.15–0.45) WCC 3 cells/ml (<5) Red cell count (RCC) 2 cells/ml (<5) Glucose 3.2 mmol/l (3.3–4.4) Blood glucose 5.8 mmol/l (3.0–6.0) Oligoclonal bands Present Serum oligoclonal bands Present Magnetic resonance scan shows a calcified lesion broadly attached to the petrous part of the temporal bone. In view of the above history and findings, what is the likely cause of this patient’s symptoms?

      Your Answer: Recurrent breast carcinoma with cerebral metastases

      Correct Answer: Meningioma of the cerebellar pontine angle

      Explanation:

      This patient has a combination of right cerebellar dysfunction with right-sided cranial nerve palsies (VI, VII, and VIII). The magnetic resonance imaging (MRI) shows a calcified meningioma within the right cerebellar pontine area, which would account for these findings. The cerebrospinal fluid (CSF) analysis shows oligoclonal bands, however, these are matched in the serum, which reflects a systemic inflammatory response from his rheumatoid arthritis.

      The MRI scan and CSF analysis would not be consistent with progressive multiple sclerosis. The progressive nature of her symptoms would be against a diagnosis of brainstem infarct, and one would expect more pyramidal signs in the peripheral nervous system.

    • This question is part of the following fields:

      • Neurology
      7.4
      Seconds
  • Question 8 - A 67-year-old butcher presents with a six month history of a gradually increasing...

    Correct

    • A 67-year-old butcher presents with a six month history of a gradually increasing burning sensation in his feet. Examination reveals normal cranial nerves and higher mental function. He has normal bulk, tone, power, light touch, pinprick sensation, co-ordination and reflexes in upper and lower limbs. These clinical findings are consistent with which of the following?

      Your Answer: Small fibre sensory neuropathy

      Explanation:

      The burning sensation described is typical of a neuropathy affecting the small unmyelinated and thinly myelinated nerve fibres. General neurological examination and reflexes are usually normal in this type of neuropathy unless there is coexisting large (myelinated) fibre involvement. Neuropathy affecting the large myelinated sensory fibres generally causes glove and stocking sensory loss and loss of reflexes.

    • This question is part of the following fields:

      • Neurology
      56
      Seconds
  • Question 9 - Causes of dilated pupils include which of the following? ...

    Incorrect

    • Causes of dilated pupils include which of the following?

      Your Answer: Pontine haemorrhage

      Correct Answer: Ethylene glycol poisoning

      Explanation:

      Ethylene glycol poisoning is the only poison listed that will cause dilation of the pupils whereas all the other listed conditions and poisons will present with small pupils other than in the case of Myotonic dystrophy wherein the patient will present with a cortical cataract.

    • This question is part of the following fields:

      • Neurology
      30.2
      Seconds
  • Question 10 - A 59-year-old scientist is referred to you with a 2-year history of ascending...

    Incorrect

    • A 59-year-old scientist is referred to you with a 2-year history of ascending lower limb numbness and, more recently, foot drop. In the last 6 months he has also developed numbness in his fingers. He has a distal reduction to pinprick and relatively preserved muscle power, except for ankle dorsiflexion and hyporeflexia in his legs. The GP has already organised nerve conduction studies and the report is sent along with the patient. Which of the following would be suggestive of an axonal neuropathy?

      Your Answer:

      Correct Answer: Reduced compound muscle action potential amplitude

      Explanation:

      Reduced conduction velocity is associated with demyelinating neuropathies. An abnormally slow response is associated with very proximal disease, i.e. radiculopathies. Delayed P100 latency is a feature of performing visual evoked potentials in those with optic nerve disease. Conduction block is usually associated with certain types of demyelinating neuropathy.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 11 - A 21-year-old patient is referred to the tertiary neurology clinic because of a...

    Incorrect

    • A 21-year-old patient is referred to the tertiary neurology clinic because of a possible diagnosis of Juvenile Parkinson’s disease. His symptoms began predominantly with dystonia affecting the lower limbs, but he now has more classical signs of older onset Parkinson’s including tremor, bradykinesia, and rigidity. You map out his family tree and understand that his sister developed Parkinson’s at the age of 16 but that his parents do not have signs of Parkinson’s. Which of the following is the most likely mode of inheritance?

      Your Answer:

      Correct Answer: Autosomal recessive

      Explanation:

      Juvenile Onset Parkinson’s is an autosomal recessive condition that usually presents in late childhood to early adulthood, initially with gait disorders caused by lower limb dystonia that later develops to the more classical signs Parkinson’s.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 12 - A 42 year old male undergoes a routine cranial nerve examination, which reveals...

    Incorrect

    • A 42 year old male undergoes a routine cranial nerve examination, which reveals the following findings: Rinne's test: Air conduction > bone conduction in both ears Weber's test: Localizes to the right side What do these test results imply?

      Your Answer:

      Correct Answer: Left sensorineural deafness

      Explanation:

      Weber’s test – if there is a sensorineural problem, the sound is localized to the unaffected side (right), indicating a problem on the left side.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 13 - A 27 year-old ballet instructor presents with 1-day history of left-sided facial weakness...

    Incorrect

    • A 27 year-old ballet instructor presents with 1-day history of left-sided facial weakness and an increased sensitivity to noise in her left ear. She is very anxious because 2 years ago she had some problems with her vision and was told that multiple sclerosis was a possibility. Her medical history is significant only for type 1 diabetes mellitus managed with insulin, and she is also taking a combined oral contraceptive pill. Upon examination, she has a lower motor neuron lesion of the left VII (facial) nerve with Bell's phenomenon present and difficulty closing her left eye. There is no objective hearing loss and no sensory signs. Examination of the auditory meatus and canal is unremarkable. The remainder of the neurological examination appears normal. The next management step in her care should be:

      Your Answer:

      Correct Answer: Eye patch and artificial tears

      Explanation:

      From the given history and physical examination findings, this patient has Bell’s palsy. There is no evidence to suggest involvement of any other cranial nerves, which might raise suspicion of a cerebello-pontine angle space-occupying lesion. With her history of possible optic neuritis, there is a possibility that the lesion is in fact a manifestation of multiple sclerosis, although this should be differentiated by examination of an upper motor neuron lesion (with sparing of the forehead facial muscles because of bilateral innervation). In light of her diabetes and the limited evidence of benefit from corticosteroid use, the most sensible first management step for her would be meticulous eye care to avoid corneal ulceration, as a result of the difficulty she is having closing her left eye.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 14 - A 44 year-old electrician is referred to you complaining of increasing problems with...

    Incorrect

    • A 44 year-old electrician is referred to you complaining of increasing problems with concentration. He also complains of irregular jerky movements of his extremities and fingers. He consumes approximately 25 units of alcohol per week. His father was diagnosed with dementia at the age of 40. Apart from generalized choreiform movements, his neurological and systemic examinations were normal. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Huntington's disease

      Explanation:

      Huntington’s disease is an autosomal inherited condition characterized by progressive dementia and worsening choreiform movements. Symptoms typically appear between ages 30 and 50. Ultimately the weakened individual succumbs to pneumonia, heart failure, or other complications.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 15 - A 52-year-old chef presents to the ED with acute visual changes. He has...

    Incorrect

    • A 52-year-old chef presents to the ED with acute visual changes. He has a past medical history of hypertension and type 2 diabetes mellitus. On neurological examination, his upper and lower limbs are normal however he has a homonymous hemianopia with central preservation. Where is the most likely cause of his problems within the central nervous system?

      Your Answer:

      Correct Answer: Optic radiation

      Explanation:

      Lesions in the optic radiation can cause a homonymous hemianopia with macular sparing, as a result of collateral circulation offered to macular tracts by the middle cerebral artery.
      Lesions in the optic tract also cause a homonymous hemianopia, but without macular sparing.
      Lesions in the optic chiasm, optic nerve, and temporal lobe cause bitemporal hemianopia, ipsilateral complete blindness, and superior homonymous quadrantanopia respectively.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 16 - A 25-year-old woman with Charcot-Marie-Tooth disease (type 1) asks how likely it is...

    Incorrect

    • A 25-year-old woman with Charcot-Marie-Tooth disease (type 1) asks how likely it is that any future children will have the disease. What is the most accurate answer?

      Your Answer:

      Correct Answer: 50%

      Explanation:

      Because Charcot-Marie-Tooth disease (type 1) is an autosomal dominant condition; therefore, there is a 50% chance that the children of this patient will be affected.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 17 - Which one of the following medications is most useful for helping to prevent...

    Incorrect

    • Which one of the following medications is most useful for helping to prevent attacks of Meniere’s disease?

      Your Answer:

      Correct Answer: Betahistine

      Explanation:

      Betahistine is a histamine analogue that has been the mainstay treatment drug for Meniere’s disease.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 18 - A 29 year-old new mother presented with a headache that was first noticed...

    Incorrect

    • A 29 year-old new mother presented with a headache that was first noticed as she was picking up her 5 week-old baby. On admission, she was unable to tolerate the lights and complained of feeling sick. Fundoscopy showed bilateral papilledema, and she was complaining that she was unable to see on her left side. CT head showed a small right occipital bleed. Which of the following treatments is most appropriate?

      Your Answer:

      Correct Answer: Heparin

      Explanation:

      This patient has developed a venous sinus thrombosis peri-partum, resulting in her symptoms. Anticoagulation therapy including Heparin improves outcomes.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 19 - A 26-year-old graduate student with a history of migraines presents for examination. His...

    Incorrect

    • A 26-year-old graduate student with a history of migraines presents for examination. His headaches are now occurring about once a week. He describes unilateral, throbbing headaches that may last over 24 hours. Neurological examination is unremarkable. Other than a history of asthma, he is fit and well. What is the most suitable therapy to reduce the frequency of migraine attacks?

      Your Answer:

      Correct Answer: Topiramate

      Explanation:

      It should be noted that as a general rule 5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor antagonists are used in prophylaxis. NICE produced guidelines in 2012 on the management of headache, including migraines. Prophylaxis should be given if patients are experiencing 2 or more attacks per month. Modern treatment is effective in about 60% of patients. NICE advises either topiramate or propranolol ‘according to the person’s preference, comorbidities and risk of adverse events’. Propranolol should be used in preference to topiramate in women of child bearing age as it may be teratogenic and it can reduce the effectiveness of hormonal contraceptives.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 20 - A 67 year-old attorney presents with a 2 month history of tremors affecting...

    Incorrect

    • A 67 year-old attorney presents with a 2 month history of tremors affecting his left arm. He suffers from depressive psychosis for the last 10 years, for which he has been receiving intermittent chlorpromazine and amitriptyline but has not been on any therapy for the last 4 months. He describes that his two brothers also had tremors. Upon examination, he has a resting tremor of his left hand with cogwheel rigidity of that arm and mild generalized bradykinesia. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Idiopathic Parkinson’s disease

      Explanation:

      The most likely diagnosis is idiopathic Parkinson’s disease because of the unilateral presentation. In addition, cogwheel rigidity is a classic presenting symptom. Neuroleptic-induced parkinsonism is usually bilateral and symmetrical. Essential tremors do not cause rest tremors.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 21 - A 75-year-old retired tailor is examined for involuntary, jerking movements of his arms....

    Incorrect

    • A 75-year-old retired tailor is examined for involuntary, jerking movements of his arms. His symptoms seem to resolve when he is asleep. Damage to which one of the following structures may lead to hemiballism?

      Your Answer:

      Correct Answer: Subthalamic nucleus

      Explanation:

      Hemiballismus or hemiballism in its unilateral form is a very rare movement disorder. It is a type of chorea caused in most cases by a decrease in activity of the subthalamic nucleus of the basal ganglia, resulting in the appearance of flailing, ballistic, undesired movements of the limbs. Symptoms may decrease while the patient is asleep. Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment.

    • This question is part of the following fields:

      • Neurology
      0
      Seconds
  • Question 22 - Which of the following is true concerning baclofen? ...

    Incorrect

    • Which of the following is true concerning baclofen?

      Your Answer:

      Correct Answer: Causes hallucinations when withdrawn

      Explanation:

      Baclofen is used to treat spastic movement symptoms such as those seen in cerebral palsy and multiple sclerosis. It is known to be associated with a withdrawal syndrome similar to alcohol withdrawal; thus, gradual withdrawal is necessary to avoid this.

    • This question is part of the following fields:

      • Neurology
      0
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  • Question 23 - A 43-year-old right-handed migraineur is admitted to hospital having developed paraesthesia affecting her...

    Incorrect

    • A 43-year-old right-handed migraineur is admitted to hospital having developed paraesthesia affecting her left arm. This came on suddenly during a migrainous attack while out shopping. The paraesthesia appeared to effect the entire left arm and in the last few hours, has spread to involve the left side of the face. She had had a similar episode several months ago whereby she developed some right-sided leg and arm weakness while at work. The weakness lasted several minutes and subsequently abated. At the time, she was also having one of her migraines. She has a long-standing history of migraines, which typically start with a prolonged aura and fortification spectra. Other then migraines, for which she has been taking pizotifen, she has no other past medical history. Her sister also suffers from migraines, and her mother has a history of dementia in her 50s. She is a non-smoker and drinks minimal alcohol. On examination she was orientated but apathetic. Her blood pressure was 130/65 mmHg, pulse 62/min, and temperature 36.2ºC. There were no carotid bruits and heart sounds were entirely normal. There was reduced sensation to all modalities over the left side of the face extending to the vertex and the entire left arm. Tone and reflexes appeared intact; however, handgrip was reduced on the left due to numbness. The lower limb appeared entirely normal. Magnetic resonance (MR) scan showed bilateral, multifocal, T2/FLAIR (fluid attenuated inversion recovery ) hyperintensities in the deep white matter. MR Spinal cord was normal. What is the diagnosis?

      Your Answer:

      Correct Answer: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy)

      Explanation:

      CADASIL is the most common form of hereditary stroke disorder. This case has a strong history of migraine with aura with stroke-like episodes, characteristic of CADASIL. Additionally, there is positive family history of migraine and early dementia but no other vascular risk factors like hypertension, diabetes, or hypercholesterolaemia, which all confirm the suspicion of CADASIL, an arteriopathy where there is thickening of the smooth muscle cells in the blood vessels.

    • This question is part of the following fields:

      • Neurology
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  • Question 24 - A 13-year-old boy complains of several episodes of collapse. He describes the majority...

    Incorrect

    • A 13-year-old boy complains of several episodes of collapse. He describes the majority of these episodes occurring when he is laughing. He states that he loses power in his legs and falls to the ground. He is alert throughout and recovers quickly. He also describes excessive daytime sleepiness with episodes in the morning of being awake but being unable to move his body. Examination is unremarkable. A diagnosis of narcolepsy is made. Which of the following is the first line treatment for excessive daytime sleepiness?

      Your Answer:

      Correct Answer: Modafinil

      Explanation:

      Narcolepsy is a rare condition characterised by excessive daytime sleepiness, sleep paralysis, hypnagogic hallucinations, and cataplexy (sudden collapse triggered by emotion such as laughing or crying). There is no cure for narcolepsy. Treatment options include stimulants, such as methylphenidate (Ritalin) or modafinil (Provigil), antidepressants, such as fluoxetine (Prozac), citalopram (Celexa), paroxetine (Paxil), sertraline (Zoloft) and sodium oxybate (Xyrem). Modafinil has replaced methylphenidate and amphetamine as the first-line treatment of excessive daytime sleepiness (EDS).

    • This question is part of the following fields:

      • Neurology
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  • Question 25 - A 73-year-old gentleman with type 2 diabetes mellitus, complains of difficulty walking and...

    Incorrect

    • A 73-year-old gentleman with type 2 diabetes mellitus, complains of difficulty walking and trouble with his hands. It began with a tingling sensation in his soles, which later extended up to his ankles. He now feels unsteady when walking, and more recently, has noticed numbness and tingling in the fingers of both hands. On examination, he has absent ankle reflexes, a high steppage gait, and altered sensation to his mid-calves. What is the underlying pathological process?

      Your Answer:

      Correct Answer: Axonal degeneration

      Explanation:

      This case presents with sensorimotor neuropathy secondary to his DM. The progression of the neuropathy, known dying-back neuropathy, is a distal axonopathy or axonal degeneration as where the sensorimotor loss begins distally and travels proximally.

    • This question is part of the following fields:

      • Neurology
      0
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  • Question 26 - A 10-year-old gentleman is referred with a six month history of daily headache,...

    Incorrect

    • A 10-year-old gentleman is referred with a six month history of daily headache, which is mostly frontal in location and occasionally associated with nausea. He has been taking paracetamol 3 g daily, aspirin 300 mg thrice daily, and codeine 40 mg thrice daily, all of which have had only a temporary effect. He has a two year history of depression treated with paroxetine. No abnormalities were found on examination. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Analgesic misuse headache

      Explanation:

      Because of the patient’s history of chronic analgesic use of daily paracetamol intake, the most likely diagnosis of this case is Analgesic misuse headache. In these cases, the headache is only temporarily relieved by analgesics. Treatment involves gradual withdrawal of analgesics.

    • This question is part of the following fields:

      • Neurology
      0
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  • Question 27 - A 58-year-old psychologist with small cell lung cancer complains of muscle weakness. Each...

    Incorrect

    • A 58-year-old psychologist with small cell lung cancer complains of muscle weakness. Each one of the following are features of Lambert-Eaton syndrome, except:

      Your Answer:

      Correct Answer: Repeated muscle contractions lead to decreased muscle strength

      Explanation:

      In myasthenia gravis, repeated muscle contractions lead to reduced muscle strength. The opposite is however classically seen in the related disorder Lambert-Eaton syndrome. Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer, and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system.

    • This question is part of the following fields:

      • Neurology
      0
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  • Question 28 - A 47-year-old hypertensive man presents with difficulty using his right arm, slow walking...

    Incorrect

    • A 47-year-old hypertensive man presents with difficulty using his right arm, slow walking and occasional loss of balance. He has a broad-based gait with cogwheel rigidity and intention tremor of his right arm. His blood pressure is 140/80 mmHg sitting and 100/60 mmHg standing. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Multiple system atrophy

      Explanation:

      This patient presents with a combination of akinetic rigid syndrome, cerebellar signs and the suggestion of autonomic features. This is most indicative of a diagnosis of multiple system atrophy.

      Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) and ataxia.

    • This question is part of the following fields:

      • Neurology
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  • Question 29 - A 69 year-old librarian with motor neuron disease is seen in clinic. Which...

    Incorrect

    • A 69 year-old librarian with motor neuron disease is seen in clinic. Which of the following interventions will have the greatest effect on survival?

      Your Answer:

      Correct Answer: Non-invasive ventilation

      Explanation:

      Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before age 40 and various patterns of disease are recognised, including amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy.
      Non-invasive ventilation (usually BIPAP) is used at night, with studies having shown a survival benefit of around 7 months. Riluzole prevents stimulation of glutamate receptors, used mainly in amyotrophic lateral sclerosis and has been shown to prolong life by about 3 months.

    • This question is part of the following fields:

      • Neurology
      0
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  • Question 30 - A 48-year-old patient with diabetes is referred from the Emergency department complaining of...

    Incorrect

    • A 48-year-old patient with diabetes is referred from the Emergency department complaining of dizziness and vomiting. On examination he is alert and orientated, his pulse is 80 irregularly irregular and BP 160/90 mmHg. There is nystagmus on left lateral gaze and his speech is slurred. On examination of the limbs, you note intention tremor and past pointing. He is ataxic when mobilised. What is the likely diagnosis?

      Your Answer:

      Correct Answer: Cerebellar CVA

      Explanation:

      The patient’s presentation with slurred speech, intention tremor and past pointing, as well as ataxia and nystagmus, paired with a history of vertigo suggest the cerebellum as the site of cerebrovascular accident (CVA) or stroke.

    • This question is part of the following fields:

      • Neurology
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  • Question 31 - A 38-year-old female patient is brought into the emergency department with a 5...

    Incorrect

    • A 38-year-old female patient is brought into the emergency department with a 5 day history of altered personality, and visual and auditory hallucinations. On palpation of the abdomen, a mass is felt in the left iliac fossa. Ultrasound of the abdomen suggests a left ovarian tumour. Her basic observations are as follows: Oxygen saturation 99% on air Heart rate 98 beats/minute Respiratory rate 28 breaths/minute Temperature 37.9 °C What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Anti-NMDA receptor encephalitis

      Explanation:

      The case presents with an underlying ovarian tumour, associated with psychiatric symptoms; thus, an organic illness must first be ruled out before considering the other conditions listed which often present with psychiatric features without an underlying organic disease. Among the listed conditions Anti-NMDA receptor encephalitis is the only condition that presents with psychiatric features including agitation, hallucinations, delusions and disordered thinking that is associated with tumours 50% of the time.

    • This question is part of the following fields:

      • Neurology
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  • Question 32 - A 6-year-old boy is referred by his GP to the neurology clinic with...

    Incorrect

    • A 6-year-old boy is referred by his GP to the neurology clinic with abnormal movements. His mother noticed that for the last year, the boy has been falling over more and more frequently. He has also been having increasingly slurred speech. These have been getting progressively worse. He has had recurrent chest infections in his childhood. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Ataxic telangiectasia

      Explanation:

      Ataxic telangiectasia is an inherited combined immunodeficiency disorder that is characterised by cerebellar ataxia and telangiectasia as seen in this child, as well as frequent infections as noted in this child’s history. The other differentials would not present with this clinical picture:

      Friedreich’s ataxia and Infantile-onset spinocerebellar ataxia do not present with immune problems, whereas Cerebral palsy and Di-George Syndrome do not present with ataxia.

    • This question is part of the following fields:

      • Neurology
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  • Question 33 - A 37-year-old teacher with multiple sclerosis complains that her vision becomes blurred during...

    Incorrect

    • A 37-year-old teacher with multiple sclerosis complains that her vision becomes blurred during a hot bath. Which of the following explain this?

      Your Answer:

      Correct Answer: Uhthoff's phenomenon

      Explanation:

      Uhthoff’s phenomenon is worsening of vision following a rise in body temperature.
      Lhermitte’s sign describes paraesthesia in the limbs on neck flexion.
      Oppenheim’s sign is seen when scratching of the inner side of leg leads to extension of the toes. It is a sign of cerebral irritation and is not related to multiple sclerosis.
      Werdnig-Hoffman’s disease is also known as spinal muscular atrophy.

    • This question is part of the following fields:

      • Neurology
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  • Question 34 - A 71-year old gentleman presents with a subacute history of intermittent difficulty in...

    Incorrect

    • A 71-year old gentleman presents with a subacute history of intermittent difficulty in walking, dry mouth, and variable slurring of speech. When the latter is severe he also has difficulty in swallowing. He has lost 3 kg in weight in the last 2 months. On examination he has bilateral mild ptosis, dysarthria, and proximal weakness of the upper and lower limbs, and he is areflexic. The degree of weakness is variable. Nerve conduction studies confirm the clinical suspicion of a neuromuscular junction disorder. Which of the following autoantibodies is likely to be the underlying cause of his neurological symptoms?

      Your Answer:

      Correct Answer: Anti-voltage-gated, calcium-channel antibody

      Explanation:

      The clinical picture points to Lambert– Eaton myasthenic syndrome (LEMS) which often presents with weakness of the arms and legs. In LEMS, antibodies against voltage-gated calcium channels (VGCC) decrease the amount of calcium that can enter the nerve ending, causing autonomic symptoms like dry mouth and slurring of speech, as seen in this patient.

    • This question is part of the following fields:

      • Neurology
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  • Question 35 - A 11-year-old is referred to neurology due to episodes her GP feels are...

    Incorrect

    • A 11-year-old is referred to neurology due to episodes her GP feels are epileptiform. Her mother reports that she appears to just 'stop', sometimes even in mid conversation, for several seconds at random times during the day. During these episodes, she can be unresponsive to questioning and has no recollection of them. Which of these drugs is contraindicated in this condition?

      Your Answer:

      Correct Answer: Carbamazepine

      Explanation:

      The patient’s history points to absence seizures. Carbamazepine has been shown to aggravate generalized seizure types, especially absence seizures, because it acts directly on the ventrobasal complex of the thalamus which is critical to the neurophysiology of absence seizures.

    • This question is part of the following fields:

      • Neurology
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  • Question 36 - Which of the following is MOST suggestive of a lesion of the sciatic...

    Incorrect

    • Which of the following is MOST suggestive of a lesion of the sciatic nerve?

      Your Answer:

      Correct Answer: Foot drop

      Explanation:

      The sciatic neve originates in the sacral plexus, mainly from spinal segments L5-S2. It supplies muscles that cause extension of the thigh and flexion of the leg. It divides into two main branches, the tibial nerve and common peroneal nerve, which are responsible for all foot movements. Anterior thigh and medial leg sensory loss is typical of a femoral nerve lesion. The femoral nerve also mediates flexion of the hip.

    • This question is part of the following fields:

      • Neurology
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  • Question 37 - A 44-year-old hairdresser with a history of myasthenia gravis is admitted to the...

    Incorrect

    • A 44-year-old hairdresser with a history of myasthenia gravis is admitted to the Emergency Department. She is currently taking pyridostigmine, but there has been a significant worsening of her symptoms following antibiotic treatment for a chest infection. On examination she is dyspnoeic and cyanotic with quiet breath sounds in both lungs. Other than respiratory support, what are the two other treatments of choice?

      Your Answer:

      Correct Answer: Plasmapheresis or intravenous immunoglobulins

      Explanation:

      This patient is having a myasthenic crisis. Opinions vary as to whether plasmapheresis or intravenous immunoglobulins should be given first-line. Plasmapheresis usually works much faster, but is more costly due to equipment.
      Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 85-90% of cases. Myasthenia gravis is more common in women (2:1).

    • This question is part of the following fields:

      • Neurology
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  • Question 38 - A 30-year-old painter presents with a burning pain in both feet, which has...

    Incorrect

    • A 30-year-old painter presents with a burning pain in both feet, which has deteriorated over the last six months. He drinks 60 units of alcohol weekly and has a family history of pernicious anaemia. On examination he has impairment of all modalities of sensation in both feet but particularly pain, temperature and absent ankle jerks. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Alcoholic peripheral neuropathy

      Explanation:

      Because of the patient’s history of excessive alcohol consumption, there is a strong suspicion of alcoholic peripheral neuropathy. In the UK, alcohol abuse and diabetes are the most common causes of peripheral neuropathy.

    • This question is part of the following fields:

      • Neurology
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  • Question 39 - A patient presented with acute onset of mild right hemiparesis affecting the body....

    Incorrect

    • A patient presented with acute onset of mild right hemiparesis affecting the body. He also has evidence of sensory loss on the right hand side. There is evidence of Horner’s syndrome and sensory loss on the face on the left hand side. Which of the following structures are involved?

      Your Answer:

      Correct Answer: Brain stem

      Explanation:

      The patient is presenting with symptoms of lateral medullary syndrome also known as Wallenberg’s syndrome or posterior inferior cerebellar artery syndrome, where the symptoms are due to an ischemia in the brainstem. The classical symptoms include contralateral sensory deficits of the trunk region paired with ipsilateral facial sensory deficits.

    • This question is part of the following fields:

      • Neurology
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  • Question 40 - A 46-year-old plumber develops chronic, severe pain after sustaining a brachial plexus injury...

    Incorrect

    • A 46-year-old plumber develops chronic, severe pain after sustaining a brachial plexus injury as a result of a motorbike accident. He has had no benefit from paracetamol or ibuprofen. In addition, he has had an unsuccessful trial of amitriptyline. Following recent NICE guidelines, which of the following is the most appropriate medication to consider?

      Your Answer:

      Correct Answer: Pregabalin

      Explanation:

      Neuropathic pain may be defined as pain which arises following damage or disruption of the nervous system. It is often difficult to treat and responds poorly to standard analgesia.
      The most recent update to the NICE guidelines for management of neuropathic pain occurred in 2013: first-line treatment* includes amitriptyline. If the first-line drug treatment does not work then move on to one of the other 3 drugs: duloxetine, gabapentin or pregabalin. Tramadol may be used as ‘rescue therapy’ for exacerbations of neuropathic pain. Topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia). Pain management clinics may be useful in patients with resistant problems.

      *please note that for some specific conditions the guidance may vary. For example carbamazepine is used first-line for trigeminal neuralgia.

    • This question is part of the following fields:

      • Neurology
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  • Question 41 - Which of the following statements regarding restless leg syndrome is not true? ...

    Incorrect

    • Which of the following statements regarding restless leg syndrome is not true?

      Your Answer:

      Correct Answer: It is three times as common in females

      Explanation:

      In restless leg syndrome (RLS), males and females are thought to be equally affected. RLS is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population and a family history may be present.

    • This question is part of the following fields:

      • Neurology
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  • Question 42 - A 32 year-old man presents with his first generalized tonic-clonic seizure (GTCS). He...

    Incorrect

    • A 32 year-old man presents with his first generalized tonic-clonic seizure (GTCS). He has been complaining of headaches for the past 2 weeks, although he has been able to continue working at his job. Upon examination, he has mild left hemiparesis and bilateral extensor plantar responses. General examination is otherwise unremarkable. An urgent CT scan of the brain shows a 5cm multicentric mass lesion in the right frontal lobe with surrounding vasogenic oedema and some hemisphere shift. Which of the following is the most likely underlying pathology?

      Your Answer:

      Correct Answer: Glioblastoma

      Explanation:

      Glioblastoma multiforme, also considered as grade IV astrocytoma, is the most malignant form of the tumour and accounts for about 20% of all cerebral tumours. These often remain clinically silent until they have reached a large enough size. In adults, glioblastoma multiforme usually occurs in the cerebral hemispheres, especially the frontal and temporal lobes of the brain. About half occupy more than one hemisphere at presentation, and some are multicentric. Biopsy shows high cellularity with mitoses, pleomorphism, and vascular hyperplasia. Prognosis is extremely poor, with only 20% surviving beyond 1 year and 10% beyond 2 years.

    • This question is part of the following fields:

      • Neurology
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  • Question 43 - A 36 year-old accountant presents with a sudden onset of headache which progressed...

    Incorrect

    • A 36 year-old accountant presents with a sudden onset of headache which progressed to him collapsing. Upon arrival in A&E, he has a heart rate of 76 bpm, blood pressure 220/140, and Glasgow Coma Score of 9 (E2, M5, V2). Which of the following should be done immediately?

      Your Answer:

      Correct Answer: Give high flow oxygen via a non-rebreather mask

      Explanation:

      This man is likely suffering from a subarachnoid haemorrhage or intracerebral bleed. The priority is to prevent a secondary brain injury. Important first steps include ensuring a secure airway, normalizing cardiovascular function, and treating seizures. His airway is likely to be protected with a GCS of 9, although he may benefit from a nasal or oral airway, and close attention should be paid to his airway if going for a CT scan. He should receive high flow oxygen and his blood pressure should not be treated acutely, as i is often appropriate to compensate for a rise in intracranial pressure. Nimodipine should be given if a subarachnoid haemorrhage is proven. Attention should also be given to maintaining a normal blood sugar, as hyperglycaemia worsens outcomes.

    • This question is part of the following fields:

      • Neurology
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  • Question 44 - Which of the following features is not associated with an oculomotor nerve palsy?...

    Incorrect

    • Which of the following features is not associated with an oculomotor nerve palsy?

      Your Answer:

      Correct Answer: Miosis

      Explanation:

      Oculomotor nerve palsy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. A complete oculomotor nerve palsy will result in a characteristic down and out position in the affected eye. This is because the lateral rectus (innervated by the sixth cranial nerve) and superior oblique (innervated by the fourth cranial or trochlear nerve), is unantagonized by the paralyzed superior rectus, inferior rectus and inferior oblique. The affected individual will also have a ptosis, or drooping of the eyelid, and mydriasis (pupil dilation), not miosis.

    • This question is part of the following fields:

      • Neurology
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  • Question 45 - A 77 year old mail carrier presents to the emergency department with severe...

    Incorrect

    • A 77 year old mail carrier presents to the emergency department with severe flinging movements of his left arm. Where would the causative lesion be located?

      Your Answer:

      Correct Answer: Right subthalamic nucleus

      Explanation:
      Hemiballismus is a hyperkinetic involuntary movement disorder characterized by intermittent, sudden, violent, involuntary, flinging, or ballistic high amplitude movements involving the ipsilateral arm and leg caused by dysfunction in the central nervous system of the contralateral side.
      Global incidence and prevalence are largely unknown, given the wide variety of etiologies but estimated to be 1-2/1,000,000, classifying it as a rare disorder.

    • This question is part of the following fields:

      • Neurology
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  • Question 46 - A 17-year-old girl presented with fever, headache and photophobia. Cerebrospinal fluid examination reveals:...

    Incorrect

    • A 17-year-old girl presented with fever, headache and photophobia. Cerebrospinal fluid examination reveals: Opening pressure 260 mm H20 (50-180) Total protein 0.8 g/l (0.15-0.45) Glucose 4.2 mmol/l (3.3-4.4) White cell count 60 per ml (<5) Lymphocytes 90% Plasma glucose 6.4 mmol/l (3.0-6.0) Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Viral meningitis

      Explanation:

      Normal cerebrospinal fluid (CSF) glucose together with lymphocytosis, an increased opening pressure and raised CSF protein are typical of a viral meningitis.

    • This question is part of the following fields:

      • Neurology
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  • Question 47 - A 38-year-old musician presented with a two-day history of sudden-onset occipital headache associated...

    Incorrect

    • A 38-year-old musician presented with a two-day history of sudden-onset occipital headache associated with nausea and vomiting. The next day, his right hand became weak for a few hours. On the same day he had an episode of sensory disturbance in his right upper limb consisting of tingling in his hand that spread up the arm, to his shoulder lasting less than two minutes in total. On the day of admission he had a similar episode of sensory disturbance lasting 30 seconds in total. On examination he had bilateral papilledema, no neck stiffness and an otherwise normal neurological examination. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Venous sinus thrombosis

      Explanation:

      Cerebral venous sinus thrombosis (CVST) is associated with headache (>90% of cases), seizures, focal weakness (40%) and papilledema (40%), all seen in this patient.
      Risk factors for CVST include genetic or acquired prothrombotic disorders, pregnancy, the oral contraceptive pill, vasculitis, malignancy, dehydration and infection. However, there are multiple other associated factors.
      Diagnosis is normally confirmed with magnetic resonance venography (MRV). Treatment is with anticoagulation, initially with heparin and subsequently with warfarin.

    • This question is part of the following fields:

      • Neurology
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  • Question 48 - A 66 year-old marketing analyst presents to the respiratory clinic with a 2-month...

    Incorrect

    • A 66 year-old marketing analyst presents to the respiratory clinic with a 2-month history of progressive weakness and shortness of breath. He finds it difficult to stand from sitting, and struggles climbing stairs. He is an ex-smoker with chronic obstructive pulmonary disease (COPD). He had a recent exacerbation one month ago for which he was treated by the GP with a course of oral prednisolone, during which time his weakness transiently improved. On examination, you note a left-sided monophonic wheeze and reduced breath sounds at the left lung base. Blood tests and a chest x-ray are requested. Hb 145 g/L WCC10.5 109/L, Na+136 mmol/L, K+ 4.3 mmol/L, Urea 6.8 mmol/L, Creatinine 93 mmol/L, Calcium 2.62 mmol/L, Phosphate 1.44 mmol/L, Chest x-ray shows hyperinflated lungs, left lower lobe collapse and a bulky left hilum What is the most likely cause of this patient's weakness?

      Your Answer:

      Correct Answer: Lambert-Eaton myasthenic syndrome

      Explanation:

      This man has a small-cell lung cancer (SCLC) and associated Lambert-Eaton myasthenic syndrome – a well-recognized paraneoplastic manifestation of SCLC. This classically affects the proximal muscles, especially in the legs, causing difficulty in standing from a seated position and climbing stairs. In contrast to myasthenia gravis, eye involvement is uncommon. Treatment with steroids is often helpful, which explains his transient symptomatic improvement during treatment for his COPD exacerbation. Steroid myopathy does not fit as the symptoms started well before his course of prednisolone. Although the patient is mildly hypercalcaemic, this would not be sufficient to produce his presenting symptoms, although it does reinforce the suspicion of lung malignancy. Motor neurone disease would be unlikely in this context and would not improve with steroids. Myasthenia gravis could produce these symptoms, but in the context of a new lung mass is a less viable diagnosis.

    • This question is part of the following fields:

      • Neurology
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  • Question 49 - A 63-year-old gentleman presents with left-sided eye pain and diplopia for the past...

    Incorrect

    • A 63-year-old gentleman presents with left-sided eye pain and diplopia for the past 2 days. Examination of his eyes shows his pupils equal and reactive to light with no proptosis. There is however an apparent palsy of the 6th cranial nerve associated with a partial 3rd nerve palsy on the left side. Examining the remaining cranial demonstrates hyperaesthesia of the upper face on the left side. Where is the likely lesion?

      Your Answer:

      Correct Answer: Cavernous sinus

      Explanation:

      A lesion on the cavernous sinus would explain the palsy observed on the III and VI cranial nerves because the cranial nerves III, IV, V, and VI pass through the cavernous sinus. Pain in the eye is due to the nearby ophthalmic veins that feeds the cavernous sinus. Additionally, the lesions in the other structures would have presented with pupil abnormalities and less localized pain and symptoms.

    • This question is part of the following fields:

      • Neurology
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  • Question 50 - A 53-year-old cashier with a history of chronic back pain presents for a...

    Incorrect

    • A 53-year-old cashier with a history of chronic back pain presents for a check-up. He is aware of a dragging feeling affecting his left foot when he tries to walk. This has developed since a minor injury to his left knee. On examination, he has weakness of dorsiflexion and eversion of the left foot. The right is unaffected and plantar flexion and inversion are normal on the left. MRI of the spinal cord shows degenerative disc changes at multiple levels, but no evidence of cord or nerve root impingement. Nerve conduction studies and EMG results are pending. Which of the following sensory loss patterns would you expect to find in association with this motor defect?

      Your Answer:

      Correct Answer: Sensory loss over the dorsum of the foot and anterolateral leg

      Explanation:

      This patient presentation is unlikely to be an L5 nerve root lesion given the results of the MRI scan. Therefore, the most likely diagnosis is a mononeuritis affecting the left common peroneal nerve. This would lead to sensory loss over the dorsum of the foot and anterolateral leg on the left.

    • This question is part of the following fields:

      • Neurology
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  • Question 51 - A 74-year-old retired judge who is known to have Alzheimer’s disease is examined...

    Incorrect

    • A 74-year-old retired judge who is known to have Alzheimer’s disease is examined in clinic. His latest Mini Mental State Examination (MMSE) score is 18 out of 30. Which of the following is the most appropriate treatment option?

      Your Answer:

      Correct Answer: Supportive care + donepezil

      Explanation:

      NICE now recommends the three acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing mild to moderate Alzheimer’s disease. Memantine is reserved for patients with moderate to severe Alzheimer’s.

    • This question is part of the following fields:

      • Neurology
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  • Question 52 - A 40 year-old lawyer suffered a road traffic accident. MRI reveals that he...

    Incorrect

    • A 40 year-old lawyer suffered a road traffic accident. MRI reveals that he may have hemisection of the spinal cord. Which of the following findings is most likely to occur?

      Your Answer:

      Correct Answer: Ipsilateral hyperreflexia

      Explanation:

      Spinal cord hemisection, also known as Brown-Sequard syndrome, is associated with symptoms affecting one spinothalamic and one corticospinal tract. Symptoms include ipsilateral paralysis, loss of vibration and position sense, and hyperreflexia below the level of the lesion. Contralateral loss of pain and temperature sensation is also seen, usually beginning 2-3 segments below the level of the lesion.

    • This question is part of the following fields:

      • Neurology
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  • Question 53 - A 51 year-old teacher presents complaining of numerous falls. He states he has...

    Incorrect

    • A 51 year-old teacher presents complaining of numerous falls. He states he has difficulty walking up stairs, and he thinks it is because of his weak legs rather than blackouts. He is hypertensive and has suffered chronic back pain for many years. He has smoked for many years as well and has a chronic smokers cough. Upon examination, he has weakness of hip flexion and particularly knee extension. He is unable to keep his fingers flexed against force, with the right being weaker than the left. There are no sensory abnormalities and reflexes are preserved bilaterally. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Inclusion body myositis

      Explanation:

      The pattern of muscle involvement seen with quadriceps and long-finger flexors is characteristic of inclusion body myositis, an inflammatory myopathy. Polymyositis is likely to cause a predominantly proximal weakness, associated with muscle pain. The signs and symptoms are not consistent with upper cord compression, as there would likely be sensory signs, reflex changes, and possible urinary symptoms. Motor neuron disease cannot be ruled out, but there are no findings of upper motor neuron or bulbar features.

    • This question is part of the following fields:

      • Neurology
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  • Question 54 - A 14 year-old is brought to the ED after being hit on the...

    Incorrect

    • A 14 year-old is brought to the ED after being hit on the head with a cricket ball during a match. His teacher describes that he initially collapsed on the ground and complained of a sore head. Two minutes later, he got up and said he felt OK and continued playing. However, 30 minutes later he suddenly collapsed and lost consciousness. What injury is he most likely to have sustained?

      Your Answer:

      Correct Answer: Extradural haematoma

      Explanation:

      A lucid interval, in which the patient portrays a temporary improvement in condition after a traumatic brain injury, is especially indicative of an epidural haematoma.

    • This question is part of the following fields:

      • Neurology
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  • Question 55 - A 61-year old mechanic recently attended A&E, with a 3 month history of...

    Incorrect

    • A 61-year old mechanic recently attended A&E, with a 3 month history of bilateral paraesthesia's and twitching affecting the thumb, first finger and lateral forearm. He denied any trauma. An MRI scan of his spine was performed and revealed cervical canal stenosis with mild cord compression. He was discharged and advised to see his GP for follow-up. Which of the following is the most appropriate initial step in management?

      Your Answer:

      Correct Answer: Refer to spinal surgery services

      Explanation:

      Bilateral median nerve dysfunction is suggestive of degenerative cervical myelopathy (DCM) rather than bilateral carpal tunnel syndrome. DCM should be suspected in elderly patients presenting with limb neurology. This patient’s twitches are probably fibrillations, a sign of lower motor neuron dysfunction.
      Degenerative cervical myelopathy is associated with a delay in diagnosis. It is most commonly misdiagnosed as carpal tunnel syndrome. In one study, 43% of patients who underwent surgery for degenerative cervical myelopathy had been initially diagnosed with carpal tunnel syndrome.
      Management of these patients should be by specialist spinal services (neurosurgery or orthopaedic spinal surgery). Decompressive surgery is the mainstay of treatment and has been shown to stop disease progression. Physiotherapy and analgesia do not replace surgical opinion, though they may be used alongside. Nerve root injections do not have a role in management.

    • This question is part of the following fields:

      • Neurology
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  • Question 56 - A 71 year-old dentist is seen in clinic with a history of worsening...

    Incorrect

    • A 71 year-old dentist is seen in clinic with a history of worsening memory problems and confusion. His wife had noted that his sleep was becoming more disturbed and he complains of vivid nightmares and visual hallucinations. Over the past few weeks, he has had increasing difficulty in dressing himself, and his mobility has deteriorated. On examination, he is bradykinesic with a resting tremor and rigidity affecting his arms and legs. His Mini-Mental-State Examination (MMSE) is 18/30. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Lewy body disease

      Explanation:

      Lewy body dementia is the second most common cause of dementia in the elderly after Alzheimer’s disease. The core feature is a progressive dementia, but other characteristic features include Parkinsonism, visual hallucinations, fluctuating cognitive abilities and executive function, and an increased risk of falls or autonomic failure.

    • This question is part of the following fields:

      • Neurology
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  • Question 57 - During the examination of an elderly confused and non-coherent gentleman who was brought...

    Incorrect

    • During the examination of an elderly confused and non-coherent gentleman who was brought to casualty by a concerned neighbour, you notice that he has bilaterally small pupils, which do not appear to react to light. Under the circumstances, it is difficult to judge their response to accommodation. Which of the following conditions may not account for the pupillary appearance in this patient?

      Your Answer:

      Correct Answer: Acute alcohol intoxication

      Explanation:

      Acute alcohol intoxication presents with pupillary dilation, thus, this is ruled out in this patient. All the other listed causes present with small pupils bilaterally.

    • This question is part of the following fields:

      • Neurology
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  • Question 58 - A 60-year-old lady has been admitted to the stroke ward following a sudden...

    Incorrect

    • A 60-year-old lady has been admitted to the stroke ward following a sudden onset of dizziness and visual disturbances, which started yesterday morning. She initially thought she was just dehydrated, however, later realised she was unable to read her own shopping list. On the ward rounds, the consultant examines her and finds she is indeed unable to read. She is, however, able to write. When she writes a sentence it makes perfect sense, although she is again unable to read it out. She has no problems with her speech and is able to converse normally. She has no motor focal neurological deficit. The consultant asks you where the lesion is likely to be?

      Your Answer:

      Correct Answer: Corpus callosum

      Explanation:

      The patient presents with sudden onset of alexia (the inability to read) WITHOUT agraphia (inability to write) which is consistent with lesions of the corpus callosum where there is a disconnect syndrome and the patient’s language and visual centres are actually in tact, but are unable to communicate between hemispheres. In this case, the damage due to the stroke is most likely in the left visual cortex, leaving visual processing to the intact right hemisphere which unfortunately cannot communicate the information to the language centres (Broca and Wernicke’s) in the left hemisphere, hence the alexia. Alternatively, the speech and writing are unaffected as the language centres can still communicate with the primary motor cortex.

    • This question is part of the following fields:

      • Neurology
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  • Question 59 - A 48-year-old hairdresser presented to her GP complaining of tingling in the right...

    Incorrect

    • A 48-year-old hairdresser presented to her GP complaining of tingling in the right palm and right wrist pain. She had intermittently experienced these symptoms; however, recently they had been keeping her awake all night. She had noticed a reduction in grip and was struggling to work at the salon. Otherwise, she felt well in herself and had not noticed any weakness in the other hand or lower limb. Her weight was stable, and she denied any neck problems or swallowing difficulties. She had a past medical history of hypothyroidism and hypertension and took regular thyroxine, Bendroflumethiazide and ibuprofen. She was a non-smoker and rarely drank alcohol. On examination, she appeared alert and orientated. Fundoscopy and cranial nerve examination were all normal and neck movements were full. On examination of the upper limb, there was wasting over the right thenar eminence and fasciculations with a small burn over the right index finger. There was weakness of thumb abduction and opposition, with loss of pinprick and light touch sensation over the thumb, index and middle finger in the right hand. Nerve conduction studies showed absent sensory action potential in right median palmar branches and denervation of the right abductor pollicis brevis. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Median nerve palsy

      Explanation:

      The history is consistent with carpal tunnel syndrome (CTS) arising as a result of pressure on the median nerve in the carpal tunnel. The median nerve supplies the muscles of the thenar eminence: the abductor pollicis (C7, C8), flexor pollicis brevis and opponens pollicis, and the lateral two lumbricals. The nerve conduction studies confirm marked denervation and absent sensory potentials within the median nerve territory.

    • This question is part of the following fields:

      • Neurology
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  • Question 60 - A 65-year-old real estate broker presents with a tremor. Which one of the...

    Incorrect

    • A 65-year-old real estate broker presents with a tremor. Which one of the following features would suggest a diagnosis of essential tremor rather than Parkinson's disease?

      Your Answer:

      Correct Answer: Tremor is worse when the arms are outstretched

      Explanation:

      Difficulty in initiating movement (bradykinesia), postural instability and unilateral symptoms (initially) are typical of Parkinson’s. Essential tremor symptoms are usually worse if arms are outstretched and eased by rest and alcohol.

    • This question is part of the following fields:

      • Neurology
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  • Question 61 - A 23-year-old designer is requesting the combined oral contraceptive pill. During the history...

    Incorrect

    • A 23-year-old designer is requesting the combined oral contraceptive pill. During the history taking, she states that in the past she has had migraines with aura. She asks why the combined oral contraceptive pill is contraindicated. Which of the following is the most appropriate response?

      Your Answer:

      Correct Answer: Significantly increased risk of ischaemic stroke

      Explanation:

      SIGN produced guidelines in 2008 on the management of migraines. Key points include that if patients have migraines with aura then the combined oral contraceptive (COC) is absolutely contraindicated due to an increased risk of stroke (relative risk 8.72).

    • This question is part of the following fields:

      • Neurology
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  • Question 62 - A 32 year-old active male presents with a three month history of pain...

    Incorrect

    • A 32 year-old active male presents with a three month history of pain in his feet and lower legs. He was previously diagnosed with diabetes at age 14 and treated with insulin. He admits to drinking 30 units of alcohol per week and is a current cannabis smoker. On examination, pain and temperature sensation in his feet are diminished, but joint position and vibratory sensation appear normal. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Diabetic polyneuropathy

      Explanation:

      The given history suggests a small fibre painful peripheral sensory neuropathy, the most common cause of which is diabetes. Joint position sense and vibratory sensation are carried through large fibres, and therefore are not currently affected. Sensory nerves are affected more often than motor, so reflexes usually remain in tact.

      Vitamin B12 deficiency causes impairment of joint position and vibratory sensation.

      Chronic inflammatory demyelinating polyneuropathy (CIPD) causes a large fibre peripheral neuropathy with areflexia.

      In syringomyelia there is impaired pain and temperature noted in the upper limbs.

      Finally, with alcoholic polyneuropathy, all fibre types are affected (sensory and motor loss). It is usually gradual with long term alcohol abuse and may be accompanied by a nutritional deficiency. In addition, pain is a more dominant feature.

    • This question is part of the following fields:

      • Neurology
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  • Question 63 - An iv-drug abuser sustains an open head injury and is admitted overnight for...

    Incorrect

    • An iv-drug abuser sustains an open head injury and is admitted overnight for observation. The next morning she is ok and is discharged. A week later she is re-admitted with fluctuating hard neurological signs. Blood results show neutrophilia and raised C-reactive protein (CRP). Which of the following is the best initial treatment step?

      Your Answer:

      Correct Answer: Computed tomography (CT) scan with contrast

      Explanation:

      Increased WBC count and CRP suggest infection. But with the fluctuating hard neurological signs, there is suspicion of the presence of a cerebral mass, which is an indication for requesting for a CT scan with contrast, to rule out an abscess or haematoma. The lumbar puncture can be considered after the CT scan.

    • This question is part of the following fields:

      • Neurology
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  • Question 64 - A 41-year-old yoga instructor presents with a 2-month history of left-hand weakness. She...

    Incorrect

    • A 41-year-old yoga instructor presents with a 2-month history of left-hand weakness. She has no significant past medical history. On examination, there is mild weakness of the left upper and lower limbs with a right sided facial weakness, which spares the forehead. Which of the following is the most likely location of the lesion?

      Your Answer:

      Correct Answer: Right pons

      Explanation:

      The pons is above the level of decussation of the corticospinal tracts so a pontine lesion would cause a contralateral limb weakness.
      The facial motor nucleus is located in the pons and supplies the ipsilateral facial muscles.
      A right cerebral lesion would give left upper and lower limb weakness. It would also cause a left sided facial weakness.
      A left cerebral lesion would give right upper and lower limb weakness with right facial weakness.
      Finally, a cervical spinal cord lesion would not cause a facial weakness.

    • This question is part of the following fields:

      • Neurology
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  • Question 65 - A 48-year-old hairdresser presents to the GP with loss of sensation over the...

    Incorrect

    • A 48-year-old hairdresser presents to the GP with loss of sensation over the lateral three and a half fingers of her right hand, tenderness over her right forearm, and inability to make a tight fist. She complains of pain in her right arm when twisting door handles anticlockwise. Phalen’s and Tinel’s tests are negative. She is otherwise neurologically intact. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Pronator teres syndrome

      Explanation:

      Entrapment of the median nerve by pronator teres causes a median nerve neuropathy, which is worse during pronation of the forearm. Examination should involve excluding carpal tunnel syndrome and pronation of the affected forearm against resistance, which brings on the pain. Unlike carpal tunnel syndrome, the median nerve proximal to the wrist may be tender to palpation.

    • This question is part of the following fields:

      • Neurology
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  • Question 66 - Three days after being admitted for a myocardial infarction, a male patient complains...

    Incorrect

    • Three days after being admitted for a myocardial infarction, a male patient complains of sudden change in vision. The medical registrar examines the patient and finds that the patient’s vision in both eyes is significantly reduced although the patient still claims that he can see. The pupils are equal in size, and the pupil responses are normal with normal fundoscopy. Significantly, the patient has now developed atrial fibrillation. A referral is made to the ophthalmologist who confirms bilateral blindness. Despite this, however, the patient fervently believes that he can see and has taken to describing objects that he has never seen previously, in discriminating detail. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Bilateral occipital cortex infarction

      Explanation:

      Bilateral occipital cortex infarction will produce varying degrees of cortical blindness, wherein the patient has no vision but fundoscopy findings are normal. When there are extensive lesions, patients my present with denial of their condition, known as Anton’s Syndrome, and begin to describe objects that they have never seen before.

    • This question is part of the following fields:

      • Neurology
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  • Question 67 - A 43 year-old female artist with no past medical history presents to the...

    Incorrect

    • A 43 year-old female artist with no past medical history presents to the emergency department with a 2-day history of pins and needles in the lower limbs, and progressive walking difficulties. She states she had diarrhoea 1 week ago. On examination, there is a loss of pinprick sensation noted to the lower limbs from mid-thigh distally and in the upper limbs from MCP joints distally. There is bilateral weakness of ankle dorsiflexion, noted at 3/5, and knee flexion and extension weakness, noted at 4/5 bilaterally. Power in upper and lower limbs is otherwise normal. Knee and ankle deep tendon reflexes are absent. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Guillain-Barre syndrome

      Explanation:

      Guillain-Barre syndrome is an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni). Characteristic features include progressive weakness of all four limbs, and it is classically ascending, affecting the lower extremities first. Sensory symptoms tend to be mild.

      Functional neurological syndrome can be discounted due to presence of hard neurological signs. Multiple sclerosis can be excluded because of the presence of lower motor neuron signs and absence of upper motor neuron signs. Chronic inflammatory demyelinating polyneuropathy is the chronic form of Guillain-Barre syndrome.

    • This question is part of the following fields:

      • Neurology
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  • Question 68 - A 20-year-old gentleman presents with drop foot following a sports injury. On examination...

    Incorrect

    • A 20-year-old gentleman presents with drop foot following a sports injury. On examination there is weakness of ankle dorsiflexion and eversion, and weakness of extension of the big toe. He has some sensory loss restricted to the dorsum of his foot surrounding the base of his big toe. Other examination is within normal limits. Where is the most likely site of the lesion?

      Your Answer:

      Correct Answer: Common peroneal nerve at the head of the fibula

      Explanation:

      Peroneal nerve injury is also known as foot drop. The common peroneal nerve supplies the ankle and toe extensor muscle groups as well as sensation over the dorsum of the foot; thus, there is also loss of sensation in these cases.

    • This question is part of the following fields:

      • Neurology
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  • Question 69 - A 20-year-old gentleman presents to the A&E department complaining of a sudden-onset occipital...

    Incorrect

    • A 20-year-old gentleman presents to the A&E department complaining of a sudden-onset occipital headache associated with vomiting. His symptoms started 2 hours previously and are continuing. He has a previous history of infrequent migraine without aura, which also causes nausea but not vomiting. He rated his current headache as much more severe than his usual migraine. Examination is unremarkable. In particular, there is no neck stiffness or photophobia. Which of the following management options would be the most appropriate?

      Your Answer:

      Correct Answer: CT brain scan, followed by lumbar puncture if CT normal

      Explanation:

      The patient presented with sudden-onset headache that is more painful than his usual migraine attacks. This gives a high suspicion of subarachnoid haemorrhage; thus, a CT brain scan should be ordered first to rule this out. However, a normal CT scan is apparent in 30% of patients with subarachnoid haemorrhage and should be referred for lumbar puncture to look for red blood cells.

    • This question is part of the following fields:

      • Neurology
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  • Question 70 - A 24-year-old gentleman presents with visual loss in his right eye, and this...

    Incorrect

    • A 24-year-old gentleman presents with visual loss in his right eye, and this is diagnosed as optic neuritis. Which one of the following statements would be seen in an afferent pupillary defect?

      Your Answer:

      Correct Answer: Accommodation response is unaffected

      Explanation:

      Afferent pupillary defect is simply a delayed pupillary response to light. Accommodation is otherwise unaffected.

    • This question is part of the following fields:

      • Neurology
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  • Question 71 - A 18-year-old gentleman is referred to dermatology. He has around 10 hyperpigmented macules...

    Incorrect

    • A 18-year-old gentleman is referred to dermatology. He has around 10 hyperpigmented macules on his torso which vary in size from 1.5-5 cm in size. His GP also noted some freckles in the groin region. He is also currently under orthopaedic review due to a worsening scoliosis of the spine. His father suffered from similar problems before having a fatal myocardial infarction two years ago. Which chromosome is most likely to have a gene defect?

      Your Answer:

      Correct Answer: Chromosome 17

      Explanation:

      The patient’s history and presentation and familial history, meets the diagnostic criteria for Neurofibromatosis type I, presenting with neurofibromas noted in this patient as hyperpigmented macules and freckles, musculoskeletal disorders like the scoliosis in this case, and a familial history. Neurofibromatosis type I is caused by a mutation on Chromosome 17.

    • This question is part of the following fields:

      • Neurology
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  • Question 72 - A 59-year-old surgeon presents with a progressive paraesthesia and numbness in both feet,...

    Incorrect

    • A 59-year-old surgeon presents with a progressive paraesthesia and numbness in both feet, which have deteriorated over the last six months. He has a 10 year history of type 2 diabetes mellitus and had cervical spondylosis, for which he underwent surgery eight years ago. He also confessed to drinking approximately 40 units of alcohol weekly. On examination he had mild bilateral weakness of foot dorsiflexion and both ankle reflexes were absent. There was absent sensation to light touch to mid-shin level with loss of joint position sensation in the toes and absent vibration sensation below the hips. He had a marked sensory ataxia and pseudoathetosis of the upper limbs. He had no evidence of a retinopathy and urinalysis was normal. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Vitamin B 12 deficiency

      Explanation:

      Diabetic peripheral neuropathy usually goes in parallel with retinopathy and nephropathy. It is also slowly progressive and affects mainly the spinothalamic pathway.
      Alcohol induced peripheral neuropathy is also slowly progressive and affects mainly the spinothalamic pathway.
      Vitamin B 12 deficiency usually causes a more rapidly progressive neuropathy with dorsal column involvement (joint position and vibration involvement with sensory ataxia and pseudoathetosis of upper limbs).

    • This question is part of the following fields:

      • Neurology
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  • Question 73 - Which one of the following is a contraindication to the use of a...

    Incorrect

    • Which one of the following is a contraindication to the use of a triptan in the management of migraine?

      Your Answer:

      Correct Answer: A history of ischaemic heart disease

      Explanation:

      A history of ischaemic heart disease in a contraindication for prescribing triptans because they act by constricting cerebral and also coronary vessels, increasing the risk of stroke.

    • This question is part of the following fields:

      • Neurology
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  • Question 74 - The chorda tympani of the facial nerve (CN VII) carries: ...

    Incorrect

    • The chorda tympani of the facial nerve (CN VII) carries:

      Your Answer:

      Correct Answer: parasympathetic fibres to the submandibular and sublingual glands and taste fibres from the anterior two-thirds of the tongue

      Explanation:

      In the petrous temporal bone the facial nerve produces three branches:
      1. The greater petrosal nerve, which transmits preganglionic parasympathetic fibres to the sphenopalatine ganglion. These postganglionic fibres supply the lacrimal gland and the glands in the nasal cavity;
      2. The nerve to stapedius;
      3. Parasympathetic fibres to the submandibular and sublingual glands and taste fibres from the anterior two-thirds of the tongue.

    • This question is part of the following fields:

      • Neurology
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  • Question 75 - An 83-year-old retired musician is examined for progressive cognitive impairment. Which one of...

    Incorrect

    • An 83-year-old retired musician is examined for progressive cognitive impairment. Which one of the following features is most suggestive of Lewy body dementia?

      Your Answer:

      Correct Answer: Symptoms worsen with neuroleptics

      Explanation:

      Lewy body dementia is an increasingly recognised cause of dementia, accounting for up to 20% of cases. The characteristic pathological feature is alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas. Neuroleptics should be avoided in Lewy body dementia as patients are extremely sensitive and may develop irreversible parkinsonism. Questions may give a history of a patient who has deteriorated following the introduction of an antipsychotic agent.

    • This question is part of the following fields:

      • Neurology
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  • Question 76 - A 26-year-old technician with no past medical history presents to the neurology clinic...

    Incorrect

    • A 26-year-old technician with no past medical history presents to the neurology clinic complaining of muscle pain. He describes severe bilateral cramping calf pain on minimal exercise. This has been present since his late teens and as such he has avoided exercise. Recently, he has attended the local gym to try and lose weight, but attempts at exercise have been dampened by the pain. He does note that if he perseveres with exercise, the pain settles. He has noticed passing dark urine in the evenings following a prolonged bout of exercise. Which of the following clinical tests would be most useful in aiding the diagnosis in this patient?

      Your Answer:

      Correct Answer: Muscle biopsy

      Explanation:

      The differential diagnosis of bilateral exercise-induced pain would include metabolic muscle disease, lumbar canal stenosis and intermittent claudication. The patient’s age, history and lack of other risk factors make the latter two options unlikely.

      The syndrome described is in fact McArdle’s disease (myophosphorylase deficiency). This is a disorder of carbohydrate metabolism. Clinical features of pain and fatigue are precipitated in early exercise, as carbohydrates cannot be mobilized to provide an energy substrate to the muscle. With prolonged exercise, fatty acid metabolism provides energy, and symptoms lessen. The dark urine described is likely to represent myoglobinuria following rhabdomyolysis. Definitive diagnosis of most metabolic muscle diseases relies on muscle biopsy and enzyme analysis.

    • This question is part of the following fields:

      • Neurology
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  • Question 77 - A 33-year-old artist who recently arrived in the UK from New York presents...

    Incorrect

    • A 33-year-old artist who recently arrived in the UK from New York presents in ED. He has a past history of insulin-dependant diabetes mellitus. He describes a few days of fever, headache and myalgia. Admission was prompted by worsening headache and back pain. While waiting in the medical receiving unit, he becomes progressively drowsier. Examination revealed flaccid paralysis and depressed tendon reflexes. He was reviewed by the intensive care team and arrangements were made for ventilation. A computerised tomography (CT) brain is performed that is normal. Cerebrospinal fluid examination reveals: Protein 0.9 g/l (<0.45 g/l) Glucose 4 mmol/L, White cell count (WCC) 28/mm3 (mostly lymphocytes) Blood testing reveals: Haemoglobin (Hb) 14 g/dl (13–18) Platelets 620 x 109/l (150–400 x 109) WCC 12 x 109/l (4–11 x 109) Sodium 135 mmol/l (137–144) Potassium 4.6 mmol/l (3.5–4.9) Urea 8 mmol/l (2.5–7.5) Creatinine 120 mmol/l (60–110) Glucose 6 mmol/L, Which of the following is the most likely infective process?

      Your Answer:

      Correct Answer: West Nile disease

      Explanation:

      West Nile virus (WNV) is a single-stranded RNA flavivirus transmitted via the culex mosquito. This previously ‘tropical’ disease has became topical in recent years following a large scale outbreak in the New York area. Incidence of neurological involvement is around 1%, although some suggest that the incidence of meningoencephalitis in America is higher than in other parts of the world. Risk factors for neurological involvement include increasing age, and immunosuppression. The usual picture is of sudden onset fever and myalgia with nausea and vomiting and a non-specific rash. Transient meningitis is occasionally seen. Frank meningoencephalitis is seen in two-thirds of cases with neurological involvement; 15% progress to coma. A flaccid paralysis similar to acute Guillain–Barré is increasingly recognised.
      Diagnosis is initially clinical with subsequent serological confirmation. Treatment is supportive; results from trials of antivirals have yielded disappointing results.

    • This question is part of the following fields:

      • Neurology
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  • Question 78 - A 41-year-old gentleman undergoes a temporal lobectomy after the discovery of a brain...

    Incorrect

    • A 41-year-old gentleman undergoes a temporal lobectomy after the discovery of a brain tumour. Which one of the following consequences would be least likely to develop?

      Your Answer:

      Correct Answer: Astereognosis

      Explanation:

      Astereognosis is associated with lesions to the parietal lobe, not the temporal lobe, so this symptom would not arise in this patient.

    • This question is part of the following fields:

      • Neurology
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  • Question 79 - A 22 year-old university graduate presented with progressive unsteadiness during walking over the...

    Incorrect

    • A 22 year-old university graduate presented with progressive unsteadiness during walking over the last year. She had been otherwise healthy apart from recent difficulty hearing her lecturer in classes. She took no prescription medication but had occasionally taken cocaine during her first year of college. She also admits to drinking up to 30 units of alcohol per week and smoked 10 cigarettes per day. Her parents were both well, but her father's sister had problems with walking before she died. Examination reveals normal tone and power throughout all four limbs. Reflexes were normal in the upper limbs but decreased at the knees and absent at the ankles. Coordination was normal in all four limbs but her gait was ataxic. Sensation in the upper limbs was normal but decreased vibratory sensation and proprioception was noted to the ankles bilaterally. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Friedreich’s ataxia

      Explanation:

      Friedreich’s ataxia is an autosomal recessive disorder that usually begins before the end of the teens. It has an estimated prevalence in Europe of 1 in 50,000 and life expectancy is around 40–50 years. Neurological features include a progressive ataxia, cerebellar dysarthria, lower limb areflexia, decreased vibratory sensation and proprioception, and pyramidal weakness. Pes cavus and scoliosis are also both seen. Cardiomyopathy occurs in over 70% of cases. Less common features include optic atrophic, diabetes mellitus, and deafness.

    • This question is part of the following fields:

      • Neurology
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  • Question 80 - A 25-year-old athlete presented with a 7-month history of difficulty gripping things. He...

    Incorrect

    • A 25-year-old athlete presented with a 7-month history of difficulty gripping things. He complained of finding it particularly difficult in cold weather. He remembered his father having similar problems. Upon examination, he had a bilateral ptosis with weakness of the facial muscles. He also had difficulty opening his eyes quickly. Limb examination revealed distal weakness in both hands with difficulty opening and closing both hands quickly. Which of the following is the most likely diagnosis?

      Your Answer:

      Correct Answer: Myotonic dystrophy

      Explanation:

      Myotonic dystrophy is the most likely diagnosis here.
      It is a multisystem disorder causing cognitive impairment, cataracts, cardiac problems and testicular atrophy, as well as affecting the muscles. Patients have muscle weakness, normally worse distally, and/or myotonia (which is worse in cold weather).
      On examination, patients may also have frontal balding, a myopathic facies, bilateral ptosis, an ophthalmoplegia and wasting of facial muscles and other limb muscles. Myotonic dystrophy is associated with diabetes mellitus and pituitary dysfunction.
      Diagnosis is normally based on clinical features with a characteristic electromyogram (EMG) of myotonic discharges. Creatine kinase is generally normal and muscle biopsy is non-specific.

    • This question is part of the following fields:

      • Neurology
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SESSION STATS - PERFORMANCE PER SPECIALTY

Neurology (5/9) 56%
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