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Question 1
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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 2
Incorrect
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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: Fasciculation of the small muscles of the hand
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.
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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 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: Parkinson’s disease
Correct Answer: Lewy body disease
Explanation:Lewy body dementia is the second most common cause of dementia in the elderly after Alzheimer’s disease. The core feature is a progressive dementia, but other characteristic features include Parkinsonism, visual hallucinations, fluctuating cognitive abilities and executive function, and an increased risk of falls or autonomic failure.
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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 51 year-old teacher presents complaining of numerous falls. He states he has difficulty walking up stairs, and he thinks it is because of his weak legs rather than blackouts. He is hypertensive and has suffered chronic back pain for many years. He has smoked for many years as well and has a chronic smokers cough. Upon examination, he has weakness of hip flexion and particularly knee extension. He is unable to keep his fingers flexed against force, with the right being weaker than the left. There are no sensory abnormalities and reflexes are preserved bilaterally. Which of the following is the most likely diagnosis?
Your Answer: Limb-girdle muscular dystrophy
Correct Answer: Inclusion body myositis
Explanation:The pattern of muscle involvement seen with quadriceps and long-finger flexors is characteristic of inclusion body myositis, an inflammatory myopathy. Polymyositis is likely to cause a predominantly proximal weakness, associated with muscle pain. The signs and symptoms are not consistent with upper cord compression, as there would likely be sensory signs, reflex changes, and possible urinary symptoms. Motor neuron disease cannot be ruled out, but there are no findings of upper motor neuron or bulbar features.
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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 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: Cerebral contusion
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 6
Correct
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A 32 year-old man presents with his first generalized tonic-clonic seizure (GTCS). He has been complaining of headaches for the past 2 weeks, although he has been able to continue working at his job. Upon examination, he has mild left hemiparesis and bilateral extensor plantar responses. General examination is otherwise unremarkable. An urgent CT scan of the brain shows a 5cm multicentric mass lesion in the right frontal lobe with surrounding vasogenic oedema and some hemisphere shift. Which of the following is the most likely underlying pathology?
Your Answer: Glioblastoma
Explanation:Glioblastoma multiforme, also considered as grade IV astrocytoma, is the most malignant form of the tumour and accounts for about 20% of all cerebral tumours. These often remain clinically silent until they have reached a large enough size. In adults, glioblastoma multiforme usually occurs in the cerebral hemispheres, especially the frontal and temporal lobes of the brain. About half occupy more than one hemisphere at presentation, and some are multicentric. Biopsy shows high cellularity with mitoses, pleomorphism, and vascular hyperplasia. Prognosis is extremely poor, with only 20% surviving beyond 1 year and 10% beyond 2 years.
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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 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 caudate nucleus
Correct Answer: Right subthalamic nucleus
Explanation:Hemiballismus is a hyperkinetic involuntary movement disorder characterized by intermittent, sudden, violent, involuntary, flinging, or ballistic high amplitude movements involving the ipsilateral arm and leg caused by dysfunction in the central nervous system of the contralateral side.Global incidence and prevalence are largely unknown, given the wide variety of etiologies but estimated to be 1-2/1,000,000, classifying it as a rare disorder. -
This question is part of the following fields:
- Neurology
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Question 8
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: Unilateral symptoms
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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Question 9
Incorrect
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A 22-year-old male has had complex tics since childhood. He repeatedly bends his knees and rubs his nose. He is prone to loud vocalisations, sometimes including swear-words. A diagnosis of Gilles de la Tourette syndrome has been made. Which of the following is the best treatment option?
Your Answer: Amitriptyline
Correct Answer: Risperidone
Explanation:Gilles de la Tourette syndrome is the most severe and rare of the tic syndromes, consisting of multiple tics involving both motor actions and vocalisation. Onset is usually in childhood. Symptoms include utterance of obscenities (coprolalia); echolalia (repetition of another person’s spoken words) and palilalia (involuntary repetition of words, phrases, or sentences).
The underlying cause is unknown, with no particular imaging or standard histopathological abnormalities having been identified. The EEG shows non-specific abnormalities in about half of patients. However, more recent immunocytochemical studies have suggested altered dopamine uptake in the striatal system.
Risperidone is an effective therapeutic option without the effects associated with chlorpromazine and haloperidol. -
This question is part of the following fields:
- Neurology
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Question 10
Correct
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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: 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 11
Incorrect
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A 63-year-old gentleman presents with left-sided eye pain and diplopia for the past 2 days. Examination of his eyes shows his pupils equal and reactive to light with no proptosis. There is however an apparent palsy of the 6th cranial nerve associated with a partial 3rd nerve palsy on the left side. Examining the remaining cranial demonstrates hyperaesthesia of the upper face on the left side. Where is the likely lesion?
Your Answer: Cerebropontine angle
Correct Answer: Cavernous sinus
Explanation:A lesion on the cavernous sinus would explain the palsy observed on the III and VI cranial nerves because the cranial nerves III, IV, V, and VI pass through the cavernous sinus. Pain in the eye is due to the nearby ophthalmic veins that feeds the cavernous sinus. Additionally, the lesions in the other structures would have presented with pupil abnormalities and less localized pain and symptoms.
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This question is part of the following fields:
- Neurology
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Question 12
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 13
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: Brainstem infarction
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 14
Correct
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A 75-year-old retired tailor is examined for involuntary, jerking movements of his arms. His symptoms seem to resolve when he is asleep. Damage to which one of the following structures may lead to hemiballism?
Your Answer: Subthalamic nucleus
Explanation:Hemiballismus or hemiballism in its unilateral form is a very rare movement disorder. It is a type of chorea caused in most cases by a decrease in activity of the subthalamic nucleus of the basal ganglia, resulting in the appearance of flailing, ballistic, undesired movements of the limbs. Symptoms may decrease while the patient is asleep. Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment.
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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 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: Ataxia telangiectasia
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 16
Correct
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A 48-year-old patient with diabetes is referred from the Emergency department complaining of dizziness and vomiting. On examination he is alert and orientated, his pulse is 80 irregularly irregular and BP 160/90 mmHg. There is nystagmus on left lateral gaze and his speech is slurred. On examination of the limbs, you note intention tremor and past pointing. He is ataxic when mobilised. What is the likely diagnosis?
Your Answer: Cerebellar CVA
Explanation:The patient’s presentation with slurred speech, intention tremor and past pointing, as well as ataxia and nystagmus, paired with a history of vertigo suggest the cerebellum as the site of cerebrovascular accident (CVA) or stroke.
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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: 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
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Question 18
Correct
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A 20-year-old gentleman presents with drop foot following a sports injury. On examination there is weakness of ankle dorsiflexion and eversion, and weakness of extension of the big toe. He has some sensory loss restricted to the dorsum of his foot surrounding the base of his big toe. Other examination is within normal limits. Where is the most likely site of the lesion?
Your Answer: Common peroneal nerve at the head of the fibula
Explanation:Peroneal nerve injury is also known as foot drop. The common peroneal nerve supplies the ankle and toe extensor muscle groups as well as sensation over the dorsum of the foot; thus, there is also loss of sensation in these cases.
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This question is part of the following fields:
- Neurology
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Question 19
Correct
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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: 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 20
Incorrect
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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: Too much cerebrospinal fluid removed
Correct 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 21
Incorrect
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Which one of the following is a contraindication to the use of a triptan in the management of migraine?
Your Answer: A history of epilepsy
Correct Answer: A history of ischaemic heart disease
Explanation:A history of ischaemic heart disease in a contraindication for prescribing triptans because they act by constricting cerebral and also coronary vessels, increasing the risk of stroke.
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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 33-year-old artist who recently arrived in the UK from New York presents in ED. He has a past history of insulin-dependant diabetes mellitus. He describes a few days of fever, headache and myalgia. Admission was prompted by worsening headache and back pain. While waiting in the medical receiving unit, he becomes progressively drowsier. Examination revealed flaccid paralysis and depressed tendon reflexes. He was reviewed by the intensive care team and arrangements were made for ventilation. A computerised tomography (CT) brain is performed that is normal. Cerebrospinal fluid examination reveals: Protein 0.9 g/l (<0.45 g/l) Glucose 4 mmol/L, White cell count (WCC) 28/mm3 (mostly lymphocytes) Blood testing reveals: Haemoglobin (Hb) 14 g/dl (13–18) Platelets 620 x 109/l (150–400 x 109) WCC 12 x 109/l (4–11 x 109) Sodium 135 mmol/l (137–144) Potassium 4.6 mmol/l (3.5–4.9) Urea 8 mmol/l (2.5–7.5) Creatinine 120 mmol/l (60–110) Glucose 6 mmol/L, Which of the following is the most likely infective process?
Your Answer: Lyme disease
Correct Answer: West Nile disease
Explanation:West Nile virus (WNV) is a single-stranded RNA flavivirus transmitted via the culex mosquito. This previously ‘tropical’ disease has became topical in recent years following a large scale outbreak in the New York area. Incidence of neurological involvement is around 1%, although some suggest that the incidence of meningoencephalitis in America is higher than in other parts of the world. Risk factors for neurological involvement include increasing age, and immunosuppression. The usual picture is of sudden onset fever and myalgia with nausea and vomiting and a non-specific rash. Transient meningitis is occasionally seen. Frank meningoencephalitis is seen in two-thirds of cases with neurological involvement; 15% progress to coma. A flaccid paralysis similar to acute Guillain–Barré is increasingly recognised.
Diagnosis is initially clinical with subsequent serological confirmation. Treatment is supportive; results from trials of antivirals have yielded disappointing results. -
This question is part of the following fields:
- Neurology
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Question 23
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: Wernicke's aphasia
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 24
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: Frontal lobe
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 25
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: Contralateral paralysis
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 26
Correct
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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: 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 27
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: 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.
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This question is part of the following fields:
- Neurology
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Question 28
Incorrect
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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 dominant with genetic anticipation
Correct Answer: Autosomal recessive
Explanation:Juvenile Onset Parkinson’s is an autosomal recessive condition that usually presents in late childhood to early adulthood, initially with gait disorders caused by lower limb dystonia that later develops to the more classical signs Parkinson’s.
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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 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: Functional neurological syndrome
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 30
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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