In hereditary sensori-motor neuropathy types i+ii (charcot-marie-tooth)
Neurology MCQs 1) In hereditary sensori-motor neuropathy types I+II (Charcot- Marie-Tooth) a) the axonal form (type II) is characterised by very slow motor nerve conduction b) the axonal form is associated with hypertrophic peripheral nerves commonly palpable clinically
c) both forms are usually inherited as an autosomal dominant condition d) pes cavus is a frequent clinical finding e) positive sensory symptoms, e.g. paraesthesia, are commonly prominent despite only minor
sensory nerve action potential disturbance
2) Autonomic neuropathy
a) is associated with a fall in systolic pressure of > 30 mmHg b) is associated with pupillary constriction with 2.5% methylcholine in each eye c) is associated with no change in heart rate when deep breathing at 6 breaths per minute d) associated with prolonged breath holding e) can be caused by Chaga’s disease
3) Acute post infective polyneuropathy (Guillain-Barré syndrome) is characteristically associated with a) an increased number of polymorphonuclear white cells in the CSF b) some disturbance of sensation c) marked muscle wasting d) reduced nerve conduction velocity e) extensor plantars 4) Diabetic neuropathies which have a good prognosis and may resolve completely include a) autonomic neuropathy b) proximal motor neuropathy c) mononeuritis involving cranial nerves d) entrapment neuropathies e) mixed sensory motor neuropathy 5) Perforating ulcers of the foot occur in
a) varicose veins b) sickle-cell disease c) tuberculoid leprosy d) diabetes mellitus e) lead neuropathy
6) Accepted features of niacin deficiency include
b) glossitis c) dermatitis d) depression e) congestive cardiac failure
7) Laurence-Moon-Biedl syndrome is characterised by
a) peripheral neuropathy b) obesity c) retinitis pigmentosa d) hyperlipaemia e) mental retardation
8) Recognised complications of chronic renal failure include
a) sensory-motor neuropathy b) pruritis c) metastatic calcification d) menorrhagia e) proximal myopathy
9) Recognised features of giant cell (cranial) arteritis include
a) male preponderance b) peripheral neuropathy c) weight loss d) myocardial ischaemia e) stroke
10) The following may be associated with hypocalcaemia a) convulsions b) psychosis c) “restless legs” d) peripheral neuropathy e) papilloedema 11) Vitamin B12 deficiency may result in a) sensory ataxia b) optic neuritis c) psychosis d) ‘glove + stocking’ anaesthesia e) increased urinary methylmalonyl CoA excretion 12) The following anatomical considerations are correct a) optic chiasma lesions characteristically produce a bitemporal hemianopia b) central scotoma occurs early in papilloedema c) in cortical blindness pupillary reactions are abnormal d) optic tract lesions produce an ipsilateral homonymous hemianopia e) opticokinetic nystagmus is found with bilateral infarction of the parieto-occipital lobes
13) Peripheral constriction of the visual fields is a recognised feature of a) optic atrophy secondary to papillaoedema b) retrobulbar neuritis c) retinitis pigmentosa d) compression of the optic chiasma e) hysterical states 14) The following are recognised manifestations of neurofibromatosis (von Recklinghausen’s disease) a) optic atrophy b) pigmentation c) paroxysms of hypertension d) albuminuria e) plexiform neuromata 15) The following neurological conditions and signs are correctly matched a) Wernike’s encephalopathy: VIth nerve palsy b) subacute combined degeneration of the spinal cord: central scotoma c) vitamin E deficiency: exagerrated tendon reflexes d) hypervitaminosis A: papilloedema e) pyridoxine toxicity: optic atrophy 16) Subacute combined degeneration of the cord a) is associated with optic atrophy b) usually presents with numbness and paraesthesia in the feet c) shows brisk abdominal reflexes d) produces motor abnormalities which resolve more completely than sensory ones e) commonly presents with a spastic paraparesis 17) A quadrantic hemianopia can arise from a) a lesion of the occipital cortex b) a lesion of the optic chiasma c) bilateral diabetic retinopathy d) tobacco amblyopia e) chloroquine poisoning 18) Complications of Behçet’s syndrome include a) thrombophlebitis b) ulcerative colitis c) erythema marginatum d) retrobulbar neuritis e) hyperuricaemia
19) Polymorphonuclear leucocytosis in the CSF may be a feature of a) carcinomatosis of the meninges b) tabes dorsalis c) acute infective polyneuritis d) tuberculous meningitis e) acute poliomyelitis 20) Decreased glucose and high polymorphonuclear cell count in the CSF may be seen in a) Echo virus meningitis b) early tuberculous meningitis c) E. coli meningitis d) meningococcal meningitis e) cryptococcal meningitis 21) A CSF protein of greater than 1.5 g/l is a characteristic feature of a) multiple sclerosis b) viral meningitis c) acoustic neuroma d) trigeminal neuralgia e) Guillain-Barre syndrome 22) Herpes simplex encephalitis a) shows a peak incidence in the Autumn b) is associated with a polymorphonuclear pleocytosis in the CSF c) produces a diffuse, evenly distributed inflammation of cerebral tissues d) produces a diagnostic EEG pattern with lateralised periodic discharges at 2 Hz e) should be treated with acyclovir as soon as the diagnosis is confirmed by urgent CSF viral 23) Hypothyroidism a) is more common than hyperthyroidism b) is associated with low serum carotene c) is a cause of pericardial effusion d) may present with cerebellar ataxia e) is associated with a reduced CSF protein 24) The following are features of encephalitits a) herpes simplex has a reasonably good prognosis in the young b) varcella zoster virus encephalitis is predominantly ‘cerebellar’ c) mumps encephalitis can cause unilateral nerve deafness d) herpes simplex predominantly affects the temporal lobe e) herpes simplex encephalitis causes a blood stained CSF 25) Which of the following may present with epilepsy? a) subdural haematoma
b) alkalosis c) paragonimiasis d) hypertension e) cadmium poisoning
26) Under the following circumstances patients with fairly well controlled epilepsy are more likely than usual to have seizures:- a) after 8 pints of beer b) while hard at work in the office c) while watching television in the evening d) two days preceding menstruation e) during a violent argument 27) Temporal lobe epilepsy is particularly associated with a) dreamy states b) an olfactory aura c) euphoria d) repetitive conjugate movements of the eyes e) visual hallucinations 28) Cerebral tumours
a) situated in the posterior fossa are more likely to be associated with epilepsy than
b) occaisionally cause overlying scalp tenderness c) are more likely to cause epilepsy if rapidly growing d) situated in the cerebellum, do not usually cause papilloedema except in terminal stages e) may cause positional vertigo if situated in the region of the fourth ventricle
29) Phenothiazine derivatives
a) have an antihistaminic effect b) act as local anaesthetics c) potentiate the effects of morphine d) may cause light hypersensitivity e) may precipitate fits in those prone to epilepsy
30) Which of the following are recognised associations with coeliac disease a) temporal lobe epilepsy b) milk intolerance c) Hashimoto’s thyroiditis d) hyposplenism e) gastric lymphoma 31) Myoclonus is caused by a) hypercalcaemia b) sodium valproate therapy
c) disorders of the olivodentate system d) subacute sclerosing panencephalitis e) epilepsy
32) Motor neurone disease a) affects men more commonly than women b) causes marked slowing of motor nerve conduction velocity c) affects the bladder and rectal sphincter in the later stages d) may be mimicked by neurosyphilis e) may cause early morning headache 33) The following cranial nerves carry pre-ganglionic parasympathetic nerves a) oculomotor nerve b) trigeminal nerve c) facial nerve d) vagus nerve e) trochlear (IV) nerve 34) The phrenic nerve a) arises predominantly from the third cervical nerve b) is a purely motor nerve c) enters the thorax lying on the lateral aspect of the vertebrae d) runs in front of the root of the lung e) innervates the diaphragm from below 35) A lesion of the sciatic nerve causes a) loss of sensation along the medial border of the foot b) loss of ankle jerk c) loss of knee jerk d) diminished power of knee flexion e) an inability to stand on the heels of the affected foot 36) The following are features of ulnar nerve entrapment at the elbow a) it occurs more commonly in women b) weakness of grip c) wasting of the thenar eminence d) symptoms and signs are more obvious when the elbow is flexed e) pain in the arm is exacerbated by repetitive supination and pronation 37) The facial nerve a) carries preganglionic parasympathetic fibres to the submandibular gland b) the nucleus receives fibres from both cerebral cortices c) supplies taste sensation to the posterior 1/3 of the tongue d) supplies motor fibres to the stapedius muscle
e) supplies touch sensation to the external auditory meatus
38) The action of noradrenaline released at sympathetic nerve endings is terminated by a) enzymatic decarboxylation b) enzymatic inactivation by catechol-O-methyl transferase c) re-uptake of noradrenaline by the axonal terminals d) oxidative deamination by monoamine oxidase e) its removal by the circulating blood 39) In a IIIrd nerve palsy there is a) ptosis b) a constricted pupil c) a convergent squint d) increased lacrimation e) enophthalmos 40) The median nerve supplies a) the lateral two interossei b) half flexor digitorum profundus c) abductor pollicis longus d) medial lumbricals e) flexor pollicis brevis 41) The ulnar nerve a) innervates the first dorsal interosseus muscle b) originates from the medial cord of the brachial plexus c) has no branches above the elbow d) innervates the medial half of the flexor digitorum profundus e) innervates the adductor pollicis muscle 42) Which of the following may cause a phrenic nerve palsy? a) aortic aneurysm b) pericardial cyst c) dermoid d) ganglioneuroma e) sarcoidosis 43) Pneumococcal meningitis
a) has its peak age incidence in childhood b) has subarachnoid block as a recognised complication c) is a recognised late sequel to splenectomy in children d) should be treated with 100 000 units benzyl penicillin intrathecally daily for seven days e) can be effectively treated with chloramphenicol in patients hypersensitive to penicillin
44) Decreased glucose and high polymorphonuclear cell count in the CSF may be seen in a) Echo virus meningitis b) early tuberculous meningitis c) E. coli meningitis d) meningococcal meningitis e) cryptococcal meningitis 45) Pyogenic meningitis
a) is commonly a result of meningococcal infection in the UK b) due to meningococcal infection is very rare in those aged less than 1 year old c) is associated with raised levels of IgM in the cerebrospinal fluid d) due to haemophilus influenzae is prone to run a subacute course in children, with the
e) is complicated by cranial nerve lesions
46) Polymorphonuclear leucocytosis in the CSF may be a feature of a) carcinomatosis of the meninges b) tabes dorsalis c) acute infective polyneuritis d) tuberculous meningitis e) acute poliomyelitis 47) Inappropriate ADH is associated with a) purulent meningitis b) head injury c) pulmonary TB d) acute intermittent porphyria e) subarachnoid haemorrhage 48) Recognised associations of Mycoplasma Pneumonia include: a) Guillan-Barre Syndrome b) Coombs positive haemolytic anaemia c) ITP d) Bullous myringitis e) cryoglobulinaemia 49) Mental retardation is an expected finding in a) glycogen storage disease b) alkaptonuria c) lactose intolerance d) maple syrup urine disease e) cystinuria 50) Parkinsonism may result from a) lead poisoning
b) Wilson’s disease c) mercury poisoning d) carbon dioxide retention e) kernicterus
51) Idiopathic hypoparathyroidism is associated with a) increased incidence of Addison’s disease b) chronic mucocutaneous candidiasis c) basal ganglia calcification, commonly causing parkinsonism d) short 4th + 5th metacarpals e) good response of hypocalcaemia to calcium and vitamin D treatment 52) Benign essential tremor
a) is worsened by alcohol b) runs in families c) is greater in the lower limbs than the upper limbs d) occurs in head e) occurs at rest
53) A man aged 40 is found to be uraemic. The following facts might give a useful lead to the aetiology a) he had haematuria as a child b) he works in an iron foundry c) three of his children had haemolytic disease of the newborn d) he has taken tablets regularly for fibrositis e) he suffers from migraine 54) Which of the following would be expected to follow a unilateral right hemi-transection of the spinal cord at L1? a) lack of pain sensation in the left ankle b) diminished 2 point discrimination on the right c) inability to determine temperature with the right foot d) a right extensor plantar response e) absent vibration sense at the right tibial tuberosity 55) Within the spinal cord a) the posterior spino-cerebellar tract carries impulses from the opposite side of the body b) the lateral spino-thalamic tract carries fibres for pain and temperature sensation c) the pyramidal tract becomes progressively larger from above downwards d) the posterior columns terminate in the gracile and cuneate nuclei e) the efferent autonomic pathway lies anterior to the pyramidal tract 56) Immediately after complete spinal cord section in the lower cervical region one would expect a) increased muscle tone in the legs b) loss of reflexes governing bladder emptying
c) increased tendon jerks d) a fall in arterial pressure e) extensor plantar responses
57) The corticospinal tract
a) runs on the anterior aspect of the medulla b) originates predominantly from the cortical cells of the precentral gyrus c) does not run in the posterior limb of the internal capsule d) runs in the pyramid e) decussates in the midbrain
58) Subacute combined degeneration of the cord a) is associated with optic atrophy b) usually presents with numbness and paraesthesia in the feet c) shows brisk abdominal reflexes d) produces motor abnormalities which resolve more completely than sensory ones e) commonly presents with a spastic paraparesis 59) Extensor plantar reflexes with absent knee jerks may occur in a) Spinal cord compresion at the 3rd and 4th lumber levels b) Friedreich’s ataxia c) pernicious anaemia d) multiple sclerosis e) taboparesis 60) The following neurological conditions and signs are correctly matched a) Wernicke’s encephalopathy: VIth nerve palsy b) subacute combined degeneration of the spinal cord: central scotoma c) vitamin E deficiency: exagerrated tendon reflexes d) hypervitaminosis A: papilloedema e) pyridoxine toxicity: optic atrophy 61) Characteristic features of Friedrich’s ataxia include a) autosomal dominant trait b) areflexia c) extensor plantar response d) hypertrophic obstructive cardiomyopathy e) DNA-triplet repeat sequences 62) Cerebellar ataxia is a recognised complication of the following a) varicella b) falciparum malaria c) paratyphoid fever d) legionnaire’s disease e) Isospora belli infection
63) Distal occlusion of the posterior cerebral artery may produce a) contralateral hemiplegia b) homonymous hemianopia c) dysarthria d) cerebellar ataxia e) palatal palsy 64) Claw feet deformities are seen with a) Wernicke’s encephalopathy b) Charcot’s arthropathy c) syringomyelia d) Friedreich’s ataxia e) peroneal muscular atrophy 65) The following are features of encephalitis a) herpes simplex has a reasonably good prognosis in the young b) varcella zoster virus encephalitis is predominantly ‘cerebellar’ c) mumps encephalitis can cause unilateral nerve deafness d) herpes simplex predominantly affects the temporal lobe e) herpes simplex encephalitis causes a blood stained CSF 66) Recurrent sterile meningitis is recognised with: a) Behcet
b) Guillain-Barre syndrome c) Sarcoidosis d) Tuberculosis e) Lepromatous
67) The following are recognised causes of bilateral lower motor neurone lesion of the seventh cranial nerve:
a) Guillain-Barre
b) Sarcoidosis c) Leprosy d) Myaesthenia gravis e) Pseudobulbar
68) The Arnold-Chiari malformation: a) Produces
b) Is often associated with syringomyelia c) Is associated with lumbosacral spina bifida d) Is associated with congenital heart lesions
69) Cerebrospinal fluid protein of 3g/litre (300mg%) is a finding in: a) Beriberi
b) General paresis of the insane c) Subdural
70) Complications of meningococcal meningitis include: a) Hydrocephalus b) Paraparesis c) Cortical 71) Retrobulbar neuritis: a) Typically produces enlargement of the blind spot b) Is caused by disseminated sclerosis c) Is cused by pentavalent tryparsamides d) Is caused by congenital toxoplasmosis e) Is caused by B12 deficiency 72) Mydriasis is found in: a) Oculomotor nerve paralysis b) Horner’s syndrome c) Retrobulbar 73) Dementia is a recognised feature of: a) Pick’s
d) Normal pressure hydrocephalus e) Motor neurone disease
Interview with Carlos Catan Antonio e Yasmin, owners of Hotel Canto das Águas, in Lençóis, Bahia (Chapada Diamantina), the first hotel in Brazil to obtain ABNT NBR 15.401 ( Accomodation – Sustainable Management System – Requirements) certification (May 2009). 1) Could you tell us a little bit about the Cato das Águas story? According to the 4 Rodas Brazil Guide, the hotel da