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Question 1
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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: Lower limb angiogram
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.
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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 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.
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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 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:
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.
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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 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:
Correct 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.
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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 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.
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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 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.
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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 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 8
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 9
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:
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 10
Incorrect
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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.
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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 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).
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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 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:
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.
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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 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.
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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 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 15
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 16
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 17
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 18
Incorrect
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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).
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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 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
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Question 20
Incorrect
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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.
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This question is part of the following fields:
- Neurology
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SESSION STATS - PERFORMANCE PER SPECIALTY
