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Question 1
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A 35-year-old woman was on a camping holiday in Spain. She awoke at three o’clock one morning with severe neck pain radiating down into her left shoulder and down to her forearm. The next day it spread to the dorsal aspect of the forearm. She was otherwise well. Her symptoms resolved after 24 hours. She noticed that after a week she was unable to wind down the car window with her left arm. On examination of the left arm there was wasting of brachioradialis, shoulder, biceps and winging of left scapula. What is the diagnosis?
Your Answer: Amyotrophic neuralgia
Explanation:This patient present with the classical symptoms of Amyotrophic neuralgia, characterised by sudden onset of pain in the shoulders that radiate down to the forearms and later resolve spontaneously but is followed by muscle wasting.
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This question is part of the following fields:
- Neurology
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Question 2
Incorrect
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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: Early onset Alzheimer's disease
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.
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This question is part of the following fields:
- Neurology
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Question 3
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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: 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 4
Incorrect
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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: Supportive care
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.
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This question is part of the following fields:
- Neurology
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Question 5
Incorrect
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Which of the following features is not associated with an oculomotor nerve palsy?
Your Answer: Pain if due to a posterior communicating artery aneurysm
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.
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This question is part of the following fields:
- Neurology
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Question 6
Correct
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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: 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 7
Incorrect
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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: Demyelination
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.
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This question is part of the following fields:
- Neurology
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Question 8
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A 21 year-old software developer undergoes a planned lumbar puncture (LP) as part of a neurological investigation for possible multiple sclerosis. During the consent process, she expresses concern about a post-LP headache. What is the mechanism of post-LP headaches?
Your Answer: Leaking cerebrospinal fluid from the dura
Explanation:Leaking of cerebrospinal fluid from the dura is the most likely explanation for post-lumbar puncture headaches. It is thought that ongoing leak of cerebrospinal fluid (CSF) through the puncture site causes ongoing CSF loss, leading to low pressure. A post-LP headache is typically frontal or occipital and occurs within three days. It is normally associated with worsening on standing and improvement when lying down. Treatment in severe cases includes an epidural blood patch, but most resolve on their own.
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This question is part of the following fields:
- Neurology
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Question 9
Incorrect
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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: Steroid-induced myopathy
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.
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This question is part of the following fields:
- Neurology
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Question 10
Correct
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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: 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.
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This question is part of the following fields:
- Neurology
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Question 11
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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.
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This question is part of the following fields:
- Neurology
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Question 12
Incorrect
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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 affecting the big toe only
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.
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This question is part of the following fields:
- Neurology
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Question 13
Incorrect
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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: Chromosome 4
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.
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This question is part of the following fields:
- Neurology
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Question 14
Incorrect
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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: Lumbar puncture
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.
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This question is part of the following fields:
- Neurology
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Question 15
Incorrect
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Which one of the following medications is most useful for helping to prevent attacks of Meniere’s disease?
Your Answer: Prochlorperazine
Correct Answer: Betahistine
Explanation:Betahistine is a histamine analogue that has been the mainstay treatment drug for Meniere’s disease.
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This question is part of the following fields:
- Neurology
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Question 16
Incorrect
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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: Botulism
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.
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This question is part of the following fields:
- Neurology
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Question 17
Incorrect
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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: Diabetic peripheral neuropathy
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 18
Correct
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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.
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This question is part of the following fields:
- Neurology
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Question 19
Incorrect
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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: Tuberculosis meningitis
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.
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This question is part of the following fields:
- Neurology
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Question 20
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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