-
Question 1
Correct
-
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: 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
-
-
Question 2
Incorrect
-
The chorda tympani of the facial nerve (CN VII) carries:
Your Answer: sympathetic fibres to the submandibular and sublingual glands and the nerve to stapedius
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
-
-
Question 3
Correct
-
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.
-
This question is part of the following fields:
- Neurology
-
-
Question 4
Incorrect
-
A 66 year-old marketing analyst presents to the respiratory clinic with a 2-month history of progressive weakness and shortness of breath. He finds it difficult to stand from sitting, and struggles climbing stairs. He is an ex-smoker with chronic obstructive pulmonary disease (COPD). He had a recent exacerbation one month ago for which he was treated by the GP with a course of oral prednisolone, during which time his weakness transiently improved. On examination, you note a left-sided monophonic wheeze and reduced breath sounds at the left lung base. Blood tests and a chest x-ray are requested. Hb 145 g/L WCC10.5 109/L, Na+136 mmol/L, K+ 4.3 mmol/L, Urea 6.8 mmol/L, Creatinine 93 mmol/L, Calcium 2.62 mmol/L, Phosphate 1.44 mmol/L, Chest x-ray shows hyperinflated lungs, left lower lobe collapse and a bulky left hilum What is the most likely cause of this patient's weakness?
Your Answer: Motor neurone disease
Correct Answer: Lambert-Eaton myasthenic syndrome
Explanation:This man has a small-cell lung cancer (SCLC) and associated Lambert-Eaton myasthenic syndrome – a well-recognized paraneoplastic manifestation of SCLC. This classically affects the proximal muscles, especially in the legs, causing difficulty in standing from a seated position and climbing stairs. In contrast to myasthenia gravis, eye involvement is uncommon. Treatment with steroids is often helpful, which explains his transient symptomatic improvement during treatment for his COPD exacerbation. Steroid myopathy does not fit as the symptoms started well before his course of prednisolone. Although the patient is mildly hypercalcaemic, this would not be sufficient to produce his presenting symptoms, although it does reinforce the suspicion of lung malignancy. Motor neurone disease would be unlikely in this context and would not improve with steroids. Myasthenia gravis could produce these symptoms, but in the context of a new lung mass is a less viable diagnosis.
-
This question is part of the following fields:
- Neurology
-
-
Question 5
Incorrect
-
Which of the following is true concerning baclofen?
Your Answer: Acts directly on skeletal muscle
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.
-
This question is part of the following fields:
- Neurology
-
-
Question 6
Correct
-
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: 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.
-
This question is part of the following fields:
- Neurology
-
-
Question 7
Correct
-
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.
-
This question is part of the following fields:
- Neurology
-
-
Question 8
Incorrect
-
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.
-
This question is part of the following fields:
- Neurology
-
-
Question 9
Incorrect
-
A 35-year-old woman was on a camping holiday in Spain. She awoke at three o’clock one morning with severe neck pain radiating down into her left shoulder and down to her forearm. The next day it spread to the dorsal aspect of the forearm. She was otherwise well. Her symptoms resolved after 24 hours. She noticed that after a week she was unable to wind down the car window with her left arm. On examination of the left arm there was wasting of brachioradialis, shoulder, biceps and winging of left scapula. What is the diagnosis?
Your Answer: syringomyelia
Correct Answer: Amyotrophic neuralgia
Explanation:This patient present with the classical symptoms of Amyotrophic neuralgia, characterised by sudden onset of pain in the shoulders that radiate down to the forearms and later resolve spontaneously but is followed by muscle wasting.
-
This question is part of the following fields:
- Neurology
-
-
Question 10
Incorrect
-
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: Cerebellar paraneoplastic syndrome
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.
-
This question is part of the following fields:
- Neurology
-
SESSION STATS - PERFORMANCE PER SPECIALTY
