IV. RESULTS
9. Characterization of c-Myb as a Cyclin O target
symptoms?
A : Pain on the outer aspect of the feet.
B : Pain radiating down both thighs C : Altered sensation in the pelvic area D : Loss of the right ankle jerk
E : Loss of anal sensation.
Comment : Outer aspect of the feet is S1 dermatome. The ankle jerk is transmitted through S1. Anal sensation is S3 dermatome. B
18. The daughter of a 62-year-old man takes him to A&E where you are asked to see him to give a medical opinion. Early that morning he developed a clumsy right hand and difficulty speaking, the problem with his hand having now persisted for 12 hours. He is known to have hypertension, asthma and rheumatoid arthritis. In addition he suffered from migraines as a young man. He smokes a pipe. On examination, he has some weakness of the intrinsic hand muscles on the right only. Visual fields, speech and sensation are normal. Blood pressure is 190/90 mmHg. Which of the following is the most likely diagnosis?
A : Left hemisphere lacunar stroke B : Migraine equivalent
C : Left middle cerebral artery territory cardioembolic stroke D : Left pontine microhaemorrhage
E : Neck-tongue syndrome.
Comment : The history suggests the dysarthria-clumsy hand syndrome, one of the classic lacunar syndromes that are strokes in the subcortical regions (or brain stem) secondary to small vessel disease.
The usual site of damage in the dysarthria-clumsy hand syndrome is the internal capsule or pons, infarction being more common than haemorrhage although both are caused by disease of small perforating arterioles. Contributory risk factors include smoking, hypertension and
hypercholesterolaemia.Migraine equivalents (aura-like symptoms without headache) can provide
diagnostic difficulties and should always be considered in those with a previous history of migraine, but there is no suggestion of the characteristic slow spread of symptoms in this patient.The neck tongue syndrome consists of pain and parasthesias in one half of the tongue precipitated by neck movement, often associated with occipital pain and ipsilateral hand parasthesias. A
19. An 84-year-old man presents with a 6-month history of increasing confusion, visual hallucinations, reduced mobility and falls. Which type of dementia fits this history best?
A : Alzheimer's disease B : Pick's disease
C : Dementia with Lewy bodies D : Parkinson's disease
E : Vascular dementia
Comment : There is some overlap between the different types of dementia, but in this case there are clues that this is dementia with Lewy bodies (DLB) with the early development of instability, falls and hallucinations. Other features include fluctuating cognition, depression and delusions.Treatment is usually symptomatic, but remember that neuroleptic drugs such as haloperidol will worsen Parkinsonian features, so consider using atypical antipsychotic agents such as quetiapine. There is some evidence that the dementia may respond to anticholinesterase inhibitors.Dementia associated with Parkinson's disease tends to occur much later in the course of the disease. C
20. A 77-year-old man presents with sudden onset of weakness of his right arm on a
background of a 3-week history of thoracolumbar backpain, weight loss, fever and night sweats.
Blood tests: full blood count (FBC) 9.8 g/dL, white cell count (WCC) 12.0, platelets 450, erythrocyte sedimentation rate (ESR) 110 mm/hr, creatinine 180 micromol/L and microscopic haematuria. What is the most likely diagnosis?
Comment : Infective endocarditis is the only diagnosis that would explain all the symptoms and results.
Vertebral osteomyelitis/discitis and stroke are both recognised complications of infective endocarditis and either can be the presenting feature of the disease. C
21. You see a 70-year-old lady who has severe Parkinson’s disease and is on co-careldopa and apomorphine. She complains of nausea and vomiting due to her medication. Which one of the following drugs would be prescribe for these symptoms?
A : Domperidone
Drugs such as apomorphine and bromocriptine cause vomiting through peripheral stimulation of the chemoreceptor trigger zone. Worsening of Parkinson’s disease may result from the use of dopamine antagonists; however, domperidone is much less likely to cross the blood–brain barrier and is therefore the preferred agent in this case. Entacapone is a catechol-O-methyltransferase (COMT) inhibitor which increases levodopa levels, thus worsening nausea and vomiting. Betahistine is used in vertigo. A
MOHAMMED IS-HAG
22. A 28-year-old male presents following an overdose and anticholinergic syndrome is suspected. Which one of the following is true?
A : Tricyclic antidepressants are not a cause B : Bradycardia is common
C : Physostigmine is the treatment of choice D : Mydriasis occurs
E : Urinary incontinence is common.
Comment :
Anticholinergic syndrome occurs following overdose with drugs that have prominent anticholinergic activity including tricyclic antidepressants, antihistamines and atropine. Features include dry, warm, flushed skin, urinary retention, tachycardia, mydriasis (dilated pupils) and agitation. Although
physostigmine, a reversible inhibitor of acteylcholinesterase, is effective in treating symptoms, there is a significant risk of cardiac toxicity (bradycardia, AV conduction defects and asystole). Treatment
therefore consists of withdrawal of the precipitating drug and supportive care. D
23. A 56-year-old woman presents with progressive leg weakness over 3 days. She has noted minor urinary incontinence in addition. She has a past medical history of breast cancer treated 10 year ago with lumpectomy and radiotherapy. Which is the investigation of choice?
A : Myelography
She is likely to have spinal cord compression from metastases. Plain radiographs and CT may give some information but the imaging modality of choice is MRI. Lumbar puncture and myelography carry the risk of clinical deterioration. E
24. A 16-year-old girl presents with a history of episodes of bizarre behaviour following her parents' recent divorce. Her mother describes these attacks to you. Which two of the following features would suggest a true epileptic seizure rather than a non-epileptic attack?
A : Limb flailing
Comment : Non-epileptic attacks can be distinguished fairly reliably on clinical grounds; however difficulty often arises with frontal lobe seizures which can be bizarre and occur without EEG change.
Cyanosis and repetitive chewing movements are more suggestive of epileptic seizures. Recovery usually occurs over a few minutes rather than immediately. Carpet burns, opisthotonus and pelvic thrusting are classically associated with non-epileptic attacks. Tongue-biting is seen with both types of attack and is non-discriminotory. G . J
25. A 43-year-old man is referred after an episode of limb paralysis occurring suddenly at nighttime. He describes waking shortly after falling asleep and being unable to move his limbs or to shout out for help. In addition he describes feeling as if he could not breathe properly.
Symptoms lasted for about a minute. His father recently died from a stroke during sleep. He has no other symptoms apart from daytime sleepiness, which he puts down to working long hours.
Blood pressure is 140/90 mmHg. There are no other abnormalities on examination. What is the most likely diagnosis?
Comment : This man is describing sleep paralysis. Sleep paralysis may be isolated, or occur in the context of narcolepsy, in which it affects 15-45% of patients. The symptoms represent the atonia of REM sleep. Awareness is preserved during the attack. Although the respiratory muscles are only ever mildly affected in comparison to the limbs, patients may describe a feeling of suffocation that can be
particularly frightening. E
26. A 29-year-old man presents with a 9-month history of depression, 4-month history of painful sensory disturbance in both legs and most recently cognitive impairment with myoclonus.
His MRI scan reveals thalamic hyperintensity on T2-weighted images. His EEG is normal. The most likely diagnosis is:
A : corticobasal degeneration
B : progressive multifocal leucoencephalopathy C : juvenile myoclonic epilepsy
D : new variant Creutzfeldt-Jakob disease
E : Wilson's disease.
Comment : New variant CJD commonly presents in young adult with psychiatric symptoms, followed by non-specific painful sensory symptoms, most often in the lower limbs. Cognitive impairment,
pyramidal signs, myoclonus and primitive reflexes then develop. Mean disease duration is approximately 14 months. MRI commonly shows high signal on T2-weighted images in the pulvinar (posterior aspect of thalamus). EEG is often normal, compared to sporadic CJD in which triphasic waves are observed.
Diagnosis is made ante-mortem by brain biopsy or more commonly tonsillar biopsy. CSF contains 14-3-3 protein. A clear link with BSE has now been established. D
27.
PLATE 26
You see a patient in clinic with the abnormality marked ‘x’ in the image. A lesion affecting which of the following nerve roots could account for the patient’s condition?
A : C4
B : C5
C : C6
D : C7
E : C8.
E :
28. A 30-year-old After 12 hours he has weakness of his arms but remains afebrile and is not confused. Over the next 12 hours he develops respiratory failure and requires artificial ventilation.
What is the likely diagnosis?
A : Tetanus
B : Diphtheria
C : Botulism
D : Strychnine poisoning
E : Rabies.
Comment : Tetanus and strychnine poisoning both produce muscle spasm which may lead to
respiratory failure but not muscle weakness.Rabies produces a uniformly fatal encephalitis characterized by pharyngeal spasm triggered by water. Diphtheria presents with a pharyngitis and a membrane over the tonsils. The toxin may cause myocarditis and neurotoxicity with palatal paralysis and cranial nerve palsies may occur. Botulism typically produces a descending paralysis which starts with diplopia or blurred vision due to difficulty with accommodation and progresses to weakness of the neck, arms and respiratory muscles. C
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29. A 40-year-old patient presents with progressive leg weakness. Which features would be against a diagnosis of Guillain–Barré syndrome?
A : Severe back pain
Comment : Guillain–Barré classically presents with an ascending paralysis beginning in the legs.
Sensory involvement is variable, but usually less than motor features. Reflexes are lost early in the illness. Demyelinating neuropathies characteristically cause fatigue with repeated use. The cranial nerves are commonly affected, most commonly the facial nerves. The autonomic nerves can be involved leading to pupillary abnormalities and haemodynamic instability. The bladder and bowel can be affected, but early involvement would be more suggestive of a conus lesion. Guillain–Barré is very painful, and can present with back pain. C.F
30. A 30-year-old woman presents with a 3-day history of left-sided retro-orbital pain and progressive visual loss. She has been previously fit and well. The following are recognised features of optic neuritis, except:
Comment : Optic neuritis presents with rapid, progressive visual loss, often associated with orbital or retro-orbital pain. Examination reveals reduced visual acuity, impaired colour vision, a central scotoma, relative afferent pupillary defect and retinal vein sheathing. It may be associated with either a normal appearing optic disc (retrobulbar neuritis) or a swollen optic disk in typical optic neuritis. Visual evoked potentials are commonly delayed. There are a number of causes including: sarcoidosis, Wegener's granulomatosis, tuberculosis, syphilis, meningitis or most commonly idiopathic (either in isolation or in association with multiple sclerosis). D.F
31. A 60-year-old alcoholic presents with a 4-week history of increasing lethargy, headache and confusion. On examination, he was confused, had signs of mild neck stiffness and photophobia.
Cerebrospinal fluid analysis revealed the following results: Opening pressure: 35 cm H2O White cells: 250 per mm3 (80% lymphocytes, 20% polymorphs) Protein: 1.5 g/l Glucose: 2.0 mmol/l (serum glucose 8.0 mmol/l) Gram stain: Negative Which of the following are the two most likely diagnoses?
Comment : The clinical features of insidious onset, meningeal irritation and cerebrospinal fluid (CSF) findings of a lymphocytic pleocytosis, high protein and low glucose make fungal and tuberculous difficult to distinguish. Therefore Ziehl-Neelsen stain, polymerase chain reaction for Mycobacterium tuberculosis, cryptococcal antigen and India ink stain should be carried out on the CSF. A chest radiograph to look for pulmonary tuberculosis will also be helpful. A.J
32. You are referred a 60-year-old man complaining of increasing difficulty with speech. Which of the following two features would make you characterize it as a posterior (receptive) type of dysphasia?
Comment : Defective voice production results in dysphonia. Impaired articulation results in dysarthria. Anterior dysphasia is characterized by non-fluent hesitant speech with an agrammatic or telepathic element, the substitution of words or syllables, poor writing with errors similar to speech, impaired naming of things and preserved comprehension, repetition and reading.
Posterior dysphasia is characterized by fluent speech with normal rhythm, neologisms, paraphrasias, substitutions and poor comprehension, repetition and reading. C.G
33. A 77-year-old woman presents with her husband who describes her dementing illness. You diagnose dementia with Lewy bodies. Which two features are the most helpful in making this diagnosis?
Comment : Dementia with Lewy bodies is probably the second commonest dementia after
Alzheimer's disease. The patients show features of dementia, with Parkinsonism, psychiatric symptoms and sometimes striking variation in performance or even conscious level. All dementias tend to produce loss of short-term memory and nocturnal wandering, and may be associated with weight loss if the patient is supposedly self-caring. Stepwise deterioration, brisk reflexes and diabetes may be associated with vascular dementia. Early impairment of language is most marked in Primary Progressive Aphasia.
Patients with progressive supranuclear palsy (PSP) are particularly prone to fall down stairs because of their impaired eye movements. B . C
34. A 22-year-old man presents with a four-day history of worsening diplopia. In the affected eye, which two of the following features would support the diagnosis of a third nerve palsy?
A : Eye is directed downwards and medially at rest B : Loss of consensual light reflex when light is shone into affected eye
C : Inward rotation of the eye on attempted down-gaze
Comment : In a third nerve palsy there is ptosis, the eye points ‘down and out’ and the pupil is dilated. On attempted downgaze the eye rotates inwards (best seen by looking at a conjunctival vessel) due to action of the superior oblique muscle (innervated by the IVth cranial nerve) on an eye that is abducted.
A fourth nerve palsy inactivates the superior oblique muscle, causing double vision with vertical
separation of images that is most marked when the patient attempts to look down, e.g. when reading or walking down stairs.
A sixth nerve palsy inactivates the lateral rectus muscle, causing double vision with horizontal
separation of images that is most apparent when the patient attempts to look to the affected side. C.F
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35. A 40-year-old man presents with a sensory peripheral neuropathy. Which two of the following are unlikely to be the cause of this man’s symptoms?
Comment : Lead toxicity and porphyria typically cause motor peripheral neuropathy. D.H 36. A 48 yr old man is referred because he has a tremor. Which one of the following features would NOT support the diagnosis of essential tremor?
A : It is an action tremor.
B : It is ameliorated by alcohol.
C : It is associated with a positive family history.
D : It has a higher frequency (Hz) than Parkinsonian tremor.
E : It is helped by anticholinergics.
Comment : Essential tremor (ET) is a common movement disorder of unknown pathogenesis. Its prevalence has been quoted to range from one to 22%, probably due to variations in definitions of ET. It is more common than Parkinson's disease (PD). It has a bimodal distribution, with peaks in the second and sixth decades of life. A family history has been reported in 17 to 100% of cases, the reason for the discrepancy being that unless all symptomatic and asymptomatic members of a family are examined, the number of affected relatives will be underestimated. ET has been mapped to several gene loci and more are expected to be identified in the future. Recently, core criteria have been suggested for the diagnosis of ET: (1) bilateral action tremor of the hands and forearms, (2) absence of other neurological signs, except the cogwheel phenomenon, and (3) may have isolated head tremor with no signs of dystonia. Secondary criteria include a long disease duration (more than three years), a positive family history and beneficial response to alcohol. Features which are against the diagnosis of ET, although not exclusively so, include unilateral, focal or leg tremor, gait disturbance, rest tremor, rigidity,
bradykinesia, sudden or rapid onset of the tremor, current drug treatment causing or exacerbating the tremor and isolated head tremor with abnormal posture (suggesting dystonia). However, there are suggestions that dystonia, parkinsonism and deafness are more common in ET. The frequency of the tremor is 4-10 Hz (compared to 4-6 Hz in PD). Propranolol and primidone are effective in the treatment of ET. If one drug does not control the tremor, a combination of both drugs may be beneficial. In severe cases refractory to medical treatment, surgical options including thalamic stimulation and thalamotomy may be needed. E
37. A 54-year-old woman presents with asymmetric hand muscle bulk. The associated clinical signs that would make you suspect a T1 root lesion include:
A : combined wasting of 1st dorsal interosseus and abductor pollicis brevis muscles
B : sensory loss over the whole palm
C : diminished finger reflexes
D : loss of pain sensation over the shoulder and across the upper chest and back E : muscle wasting in shoulder girdle
Comment : A T1 root lesion will lead to wasting of all intrinsic hand muscles, diminished sensation along the medial aspect of the arm, and will be associated with a Horner’s syndrome. In all such patients a chest radiograph is mandatory, whether they are smokers or not, although this would heighten your suspicion that an apical lung tumour has caused the root compression.
Sensory loss over the whole palm in this situation would suggest combined ulnar and median nerve involvement, and the dissociated -cape distribution sensory loss is seen in intrinsic cord lesions at around T1, e.g. syringomyelia. The muscle wasting in this diagnosis is caused by compression of the anterior horn cells by the syrinx. Finger reflexes are a C8 reflex, but are most useful when brisk, rather than absent, which is difficult to distinguish from normal.
An inverted biceps reflex is a feature of a cord lesion at C5,6. A.F
38. A 60-year-old man is found to have a parietal lobe tumour. Which of the following would
Comment : Common presentations of a parietal lobe tumour include contralateral sensory or cortical sensory loss, neglect, apraxia and contralateral homonymous field defect (that sometimes consists solely of lower quadrantanopia). D.G
39. You are asked to see a 36-year-old woman with headaches. Which two of the following features would suggest a diagnosis of tension-type headache?
A : Aggravated by physical activity
Comment :Tension-type headache is characterized by a constant, non-pulsatile, band-like, bi-fronto-temporal pressure on top of the head. The headache is not worsened by physical activity. Associated mild nausea, but not vomiting, is common. Chronic forms are worsened by anxiety and stress. Alcohol may relieve the headache. Regular simple analgesia may be the commonest cause of chronic daily headache. There are no abnormal features on examination. Amitryptyline is the drug treatment of choice. E .G
40. A 72-year-old man is referred with suspected Parkinson’s disease. He complains of shaking of his hands which is worse when he tries to do things such as drinking a cup of tea, which has got progressively worse over the last 5 years. He comments that his father had a similar condition.
He is otherwise well and has a normal posture and gait. You diagnose benign essential tremor.
Which two statements about this condition are correct?
A : The tremor is an intention tremor.
B : Drugs such as sodium valproate and lithium may produce a similar tremor.
C : In young people, Wilson's disease, indicated by a high serum caeruloplasmin, should be
excluded.
D : Many cases are familial with autosomal recessive inheritance.
E : This is a clinical diagnosis and no baseline investigation is required.
F : Medication should always be offered since it may halt progression of the condition.
G : Primidone is well tolerated initially but has a high rate of long-term complications.
H : Gabapentin, a GABA-antagonist, is a useful second-line agent.
I : Over 90% of patients will have marked improvement with pharmacotherapy therapy.
J : Thalamic stimulation may be recommended in those with disabling tremor unresponsive to
medication.
Comment : Benign (a misnomer since it can be disabling ) essential tremor (BET) predominantly affects those over the age of 70. The tremor is pathological, unlike the physiological tremor we all have which may become noticeable in stressful situations. It is an ACTION tremor which occurs during
Comment : Benign (a misnomer since it can be disabling ) essential tremor (BET) predominantly affects those over the age of 70. The tremor is pathological, unlike the physiological tremor we all have which may become noticeable in stressful situations. It is an ACTION tremor which occurs during