-
Question 1
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: Fluoxetine
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
-
-
Question 2
Incorrect
-
A 39 year-old engineer presents with progressive weakness of his hands. Upon examination, you notice wasting of the small muscles of the hand. A diagnosis of syringomyelia is suspected. Which one of the following features would most support this diagnosis?
Your Answer: Hyper-reflexia in the upper limbs
Correct Answer: Loss of temperature sensation in the hands
Explanation:Syringomyelia is a development of a cavity (syrinx) within the spinal cord. Signs and symptoms include loss of feeling, paralysis, weakness, and stiffness in the back, shoulders, and extremities. Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold, especially in the hands. Symptoms typically vary depending on the extent and, often more critically, on the location of the syrinx within the spinal cord.
-
This question is part of the following fields:
- Neurology
-
-
Question 3
Correct
-
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: 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
-
-
Question 4
Incorrect
-
Which of the following types of motor neuron diseases carries the worst prognosis?
Your Answer: Relapsing-remitting
Correct 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
-
-
Question 5
Correct
-
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: 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
-
-
Question 6
Incorrect
-
A 78-year-old retired journalist known to have prostatic carcinoma presents to the ED complaining of pain in the spine and the onset of severe lower-leg weakness accompanied by a loss of sensation. On examination, he is found to have percussion tenderness of his spine, loss of sensation up to the umbilicus and a distended bladder. He has markedly reduced power of the lower legs with hyperreflexia. Which of the following should not be part of your management of this patient?
Your Answer: Magnetic resonance imaging (MRI) of the spinal column
Correct Answer: Spinal X-rays
Explanation:Acute cord compression is a medical emergency. Typically, signs of segmental damage at the level of compression are usually combined with corticospinal tract dysfunction (e.g., hyperreflexia, Babinski’s sign and weakness) and sensory deficits below the level of compression. Symptoms include spinal pain that precedes the development of weak legs and sensory loss. There may be loss of bladder (and anal) sphincter control, manifesting as hesitancy, frequency and, finally, painless retention.
Spinal X-rays are rarely diagnostic. MRI is usually the investigation of choice and should not be delayed, but if not available consider doing a CT scan and myelography to confirm cord compression and fully define the level and extent of the lesion. If malignancy is the cause, it is important to give dexamethasone (oral or intravenous) while considering therapy more specific to the cause. -
This question is part of the following fields:
- Neurology
-
-
Question 7
Correct
-
A 72-year-old woman presents with 18 month history of gait ataxia, dysarthria, and dysphagia. On examination there is down beating nystagmus and slurred speech. There is past pointing in both upper limbs and a wide-based ataxic gait. Reflexes and sensation are normal. There is no wasting or fasciculations. Plantar response is flexor bilaterally. What is the most likely diagnosis?
Your Answer: Arnold-Chiari malformation
Explanation:Downbeat nystagmus (DBN) suggests a lesion in the lower part of the medulla. Arnold-Chiari malformation usually presents with symptoms due to brainstem and lower cranial nerve dysfunction such as DBN.
-
This question is part of the following fields:
- Neurology
-
-
Question 8
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: Dexamethasone iv
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
-
-
Question 9
Correct
-
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: 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
-
-
Question 10
Correct
-
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: 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
-
-
Question 11
Correct
-
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: 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
-
-
Question 12
Correct
-
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: 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
-
-
Question 13
Correct
-
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: 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
-
-
Question 14
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: Reduced conduction velocity
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
-
-
Question 15
Correct
-
Which of the following peripheral neuropathy inducing clinical diagnoses is predominantly associated with sensory loss?
Your 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
-
-
Question 16
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: Cerebellar paraneoplastic syndrome
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
-
-
Question 17
Correct
-
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: 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
-
-
Question 18
Correct
-
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: 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
-
-
Question 19
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: C8
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
-
-
Question 20
Correct
-
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: 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
-
SESSION STATS - PERFORMANCE PER SPECIALTY
