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Question 1
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: Occipital 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 2
Incorrect
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A 47-year-old hypertensive man presents with difficulty using his right arm, slow walking and occasional loss of balance. He has a broad-based gait with cogwheel rigidity and intention tremor of his right arm. His blood pressure is 140/80 mmHg sitting and 100/60 mmHg standing. Which of the following is the most likely diagnosis?
Your Answer: Idiopathic Parkinson’s disease
Correct Answer: Multiple system atrophy
Explanation:This patient presents with a combination of akinetic rigid syndrome, cerebellar signs and the suggestion of autonomic features. This is most indicative of a diagnosis of multiple system atrophy.
Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) and ataxia.
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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 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: Duchenne’s muscular dystrophy
Correct 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 4
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: Chronic inflammatory demyelinating polyradiculopathy
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 5
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 6
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: Antiganglioside antibody GQ1B
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 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: Globus pallidus
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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Which of the following peripheral neuropathy inducing clinical diagnoses is predominantly associated with sensory loss?
Your Answer: Hereditary sensorimotor neuropathies
Correct Answer: Uraemia
Explanation:Diseases that cause predominantly sensory loss include diabetes, uraemia, leprosy, alcoholism, vitamin B12 deficiency, and amyloidosis.
Those that cause predominantly motor loss include Guillain-Barre syndrome, porphyria, lead poisoning, hereditary sensorimotor neuropathies, chronic inflammatory demyelinating polyneuropathy, and diphtheria. -
This question is part of the following fields:
- Neurology
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Question 9
Correct
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Which of the following statements regarding restless leg syndrome is not true?
Your 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 10
Correct
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A 48-year-old hairdresser presented to her GP complaining of tingling in the right palm and right wrist pain. She had intermittently experienced these symptoms; however, recently they had been keeping her awake all night. She had noticed a reduction in grip and was struggling to work at the salon. Otherwise, she felt well in herself and had not noticed any weakness in the other hand or lower limb. Her weight was stable, and she denied any neck problems or swallowing difficulties. She had a past medical history of hypothyroidism and hypertension and took regular thyroxine, Bendroflumethiazide and ibuprofen. She was a non-smoker and rarely drank alcohol. On examination, she appeared alert and orientated. Fundoscopy and cranial nerve examination were all normal and neck movements were full. On examination of the upper limb, there was wasting over the right thenar eminence and fasciculations with a small burn over the right index finger. There was weakness of thumb abduction and opposition, with loss of pinprick and light touch sensation over the thumb, index and middle finger in the right hand. Nerve conduction studies showed absent sensory action potential in right median palmar branches and denervation of the right abductor pollicis brevis. What is the most likely diagnosis?
Your Answer: Median nerve palsy
Explanation:The history is consistent with carpal tunnel syndrome (CTS) arising as a result of pressure on the median nerve in the carpal tunnel. The median nerve supplies the muscles of the thenar eminence: the abductor pollicis (C7, C8), flexor pollicis brevis and opponens pollicis, and the lateral two lumbricals. The nerve conduction studies confirm marked denervation and absent sensory potentials within the median nerve territory.
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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 11-year-old is referred to neurology due to episodes her GP feels are epileptiform. Her mother reports that she appears to just 'stop', sometimes even in mid conversation, for several seconds at random times during the day. During these episodes, she can be unresponsive to questioning and has no recollection of them. Which of these drugs is contraindicated in this condition?
Your Answer: Clonazepam
Correct Answer: Carbamazepine
Explanation:The patient’s history points to absence seizures. Carbamazepine has been shown to aggravate generalized seizure types, especially absence seizures, because it acts directly on the ventrobasal complex of the thalamus which is critical to the neurophysiology of absence seizures.
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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 25-year-old woman with Charcot-Marie-Tooth disease (type 1) asks how likely it is that any future children will have the disease. What is the most accurate answer?
Your Answer: 50%
Explanation:Because Charcot-Marie-Tooth disease (type 1) is an autosomal dominant condition; therefore, there is a 50% chance that the children of this patient will be affected.
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This question is part of the following fields:
- Neurology
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Question 13
Incorrect
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During the examination of an elderly confused and non-coherent gentleman who was brought to casualty by a concerned neighbour, you notice that he has bilaterally small pupils, which do not appear to react to light. Under the circumstances, it is difficult to judge their response to accommodation. Which of the following conditions may not account for the pupillary appearance in this patient?
Your Answer: Diabetes mellitus
Correct Answer: Acute alcohol intoxication
Explanation:Acute alcohol intoxication presents with pupillary dilation, thus, this is ruled out in this patient. All the other listed causes present with small pupils bilaterally.
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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 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: Chronic inflammatory demyelinating polyneuropathy
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 15
Correct
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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: 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 16
Correct
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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: 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 17
Incorrect
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A 27 year-old ballet instructor presents with 1-day history of left-sided facial weakness and an increased sensitivity to noise in her left ear. She is very anxious because 2 years ago she had some problems with her vision and was told that multiple sclerosis was a possibility. Her medical history is significant only for type 1 diabetes mellitus managed with insulin, and she is also taking a combined oral contraceptive pill. Upon examination, she has a lower motor neuron lesion of the left VII (facial) nerve with Bell's phenomenon present and difficulty closing her left eye. There is no objective hearing loss and no sensory signs. Examination of the auditory meatus and canal is unremarkable. The remainder of the neurological examination appears normal. The next management step in her care should be:
Your Answer: Eighty mg oral prednisolone for five days
Correct Answer: Eye patch and artificial tears
Explanation:From the given history and physical examination findings, this patient has Bell’s palsy. There is no evidence to suggest involvement of any other cranial nerves, which might raise suspicion of a cerebello-pontine angle space-occupying lesion. With her history of possible optic neuritis, there is a possibility that the lesion is in fact a manifestation of multiple sclerosis, although this should be differentiated by examination of an upper motor neuron lesion (with sparing of the forehead facial muscles because of bilateral innervation). In light of her diabetes and the limited evidence of benefit from corticosteroid use, the most sensible first management step for her would be meticulous eye care to avoid corneal ulceration, as a result of the difficulty she is having closing her left eye.
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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 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 19
Correct
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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: 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 20
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: Regular chest physiotherapy
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 21
Incorrect
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The chorda tympani of the facial nerve (CN VII) carries:
Your Answer: sympathetic fibres to the submandibular and sublingual glands and taste fibres from the anterior two-thirds of the tongue
Correct Answer: parasympathetic fibres to the submandibular and sublingual glands and taste fibres from the anterior two-thirds of the tongue
Explanation:In the petrous temporal bone the facial nerve produces three branches:
1. The greater petrosal nerve, which transmits preganglionic parasympathetic fibres to the sphenopalatine ganglion. These postganglionic fibres supply the lacrimal gland and the glands in the nasal cavity;
2. The nerve to stapedius;
3. Parasympathetic fibres to the submandibular and sublingual glands and taste fibres from the anterior two-thirds of the tongue. -
This question is part of the following fields:
- Neurology
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Question 22
Correct
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A 67 year-old attorney presents with a 2 month history of tremors affecting his left arm. He suffers from depressive psychosis for the last 10 years, for which he has been receiving intermittent chlorpromazine and amitriptyline but has not been on any therapy for the last 4 months. He describes that his two brothers also had tremors. Upon examination, he has a resting tremor of his left hand with cogwheel rigidity of that arm and mild generalized bradykinesia. Which of the following is the most likely diagnosis?
Your Answer: Idiopathic Parkinson’s disease
Explanation:The most likely diagnosis is idiopathic Parkinson’s disease because of the unilateral presentation. In addition, cogwheel rigidity is a classic presenting symptom. Neuroleptic-induced parkinsonism is usually bilateral and symmetrical. Essential tremors do not cause rest tremors.
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This question is part of the following fields:
- Neurology
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Question 23
Correct
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A 38-year-old female patient is brought into the emergency department with a 5 day history of altered personality, and visual and auditory hallucinations. On palpation of the abdomen, a mass is felt in the left iliac fossa. Ultrasound of the abdomen suggests a left ovarian tumour. Her basic observations are as follows: Oxygen saturation 99% on air Heart rate 98 beats/minute Respiratory rate 28 breaths/minute Temperature 37.9 °C What is the most likely diagnosis?
Your Answer: Anti-NMDA receptor encephalitis
Explanation:The case presents with an underlying ovarian tumour, associated with psychiatric symptoms; thus, an organic illness must first be ruled out before considering the other conditions listed which often present with psychiatric features without an underlying organic disease. Among the listed conditions Anti-NMDA receptor encephalitis is the only condition that presents with psychiatric features including agitation, hallucinations, delusions and disordered thinking that is associated with tumours 50% of the time.
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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 59-year-old scientist is referred to you with a 2-year history of ascending lower limb numbness and, more recently, foot drop. In the last 6 months he has also developed numbness in his fingers. He has a distal reduction to pinprick and relatively preserved muscle power, except for ankle dorsiflexion and hyporeflexia in his legs. The GP has already organised nerve conduction studies and the report is sent along with the patient. Which of the following would be suggestive of an axonal neuropathy?
Your Answer: Reduced conduction velocity
Correct Answer: Reduced compound muscle action potential amplitude
Explanation:Reduced conduction velocity is associated with demyelinating neuropathies. An abnormally slow response is associated with very proximal disease, i.e. radiculopathies. Delayed P100 latency is a feature of performing visual evoked potentials in those with optic nerve disease. Conduction block is usually associated with certain types of demyelinating neuropathy.
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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 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: Pupil of affected eye larger than the unaffected eye
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 26
Incorrect
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Which of the following is true concerning baclofen?
Your Answer: Reduces Ca2+ release from sarcoplasmic reticulum
Correct Answer: Causes hallucinations when withdrawn
Explanation:Baclofen is used to treat spastic movement symptoms such as those seen in cerebral palsy and multiple sclerosis. It is known to be associated with a withdrawal syndrome similar to alcohol withdrawal; thus, gradual withdrawal is necessary to avoid this.
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This question is part of the following fields:
- Neurology
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Question 27
Incorrect
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Causes of dilated pupils include which of the following?
Your Answer: Argyll Robertson pupil
Correct Answer: Ethylene glycol poisoning
Explanation:Ethylene glycol poisoning is the only poison listed that will cause dilation of the pupils whereas all the other listed conditions and poisons will present with small pupils other than in the case of Myotonic dystrophy wherein the patient will present with a cortical cataract.
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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 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: Dexamphetamine
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 29
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: Haloperidol
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 30
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: Primary progressive multiple sclerosis
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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SESSION STATS - PERFORMANCE PER SPECIALTY
