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Question 1
Correct
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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: 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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Question 2
Incorrect
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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:
Correct 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.
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This question is part of the following fields:
- Neurology
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Question 3
Incorrect
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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:
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.
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This question is part of the following fields:
- Neurology
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Question 4
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 5
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 6
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 7
Incorrect
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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
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Question 8
Incorrect
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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.
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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 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.
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This question is part of the following fields:
- Neurology
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Question 10
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:
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 11
Incorrect
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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:
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.
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This question is part of the following fields:
- Neurology
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Question 12
Incorrect
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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.
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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 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.
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This question is part of the following fields:
- Neurology
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Question 14
Incorrect
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A 27-year-old realtor presented with progressive weakness of both legs over the last 3 years. He complained of being unable to see well at night and having an impaired sense of smell. On examination he had a shortened fourth toe bilaterally with pes cavus. Neurological examination revealed a loss of pinprick sensation to bilateral knees, and weakness of both legs that was more prominent distally. Which of the following would be the best blood test to order to make a diagnosis?
Your Answer:
Correct Answer: Phytanic acid
Explanation:The diagnosis is Refsum’s disease. This is an autosomal recessive disorder that causes a sensorimotor peripheral neuropathy. It is caused by defective alpha oxidation of phytanic acid leading to its accumulation in tissues. Cardiac conduction abnormalities and cardiomyopathies may also occur.
Epiphyseal dysplasia causes a characteristic shortening of the fourth toe. Serum phytanic acid levels are elevated. Treatment is by dietary restriction of foods containing phytanic acid (dairy products, fish, beef and lamb). -
This question is part of the following fields:
- Neurology
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Question 15
Incorrect
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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 16
Incorrect
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Which of the following types of motor neuron diseases carries the worst prognosis?
Your Answer:
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.
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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 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.
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This question is part of the following fields:
- Neurology
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Question 18
Incorrect
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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:
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.
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This question is part of the following fields:
- Neurology
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Question 19
Incorrect
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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.
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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 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.
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This question is part of the following fields:
- Neurology
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Question 21
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 22
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 23
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:
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 24
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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Question 25
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 26
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 27
Incorrect
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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:
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
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Question 28
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 29
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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Question 30
Incorrect
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A 27-year-old gentleman presents with a 2 year history of involuntary neck movements. There is no history of trauma. He is on no drugs. There is no relevant family history. On examination he has an episodic right torticollis. The rest of his neurological examination is normal. MRI of his cervical spine is normal. The neurologist in the Movement Disorders Clinic has diagnosed cervical dystonia. Which of the following treatments will be most beneficial?
Your Answer:
Correct Answer: Botulinum toxin
Explanation:Botulinum toxin is a neurotoxic protein from Clostridium botulinum that causes flaccid paralysis as it acts by preventing the release of Ach at the neuromuscular joint. It is the first-line treatment for cervical dystonia (torticollis) because the condition is a neurological disorder characterised by unusual muscle contractions of the neck. With the use of Botulinum toxin, the contractions would be released.
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This question is part of the following fields:
- Neurology
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