Neurological Board Examination (II)

Transcription

1 Neurological Board Examination (II) B-type: For each numbered item, select the heading most closely associated with it. Each heading may be selected once, more than once, or not at all Part 1 (1-2) ( )1. Cat-Scratch encephalitis ( )2. Lyme disease A. Tropheryma whippelii B. Bartonella henselae C. Borrelia burgdorferi Part 2 (3-4) 下列疾病可能與何離子通道之基因突變有關 ( )3. Periodic paralysis ( )4. Myotonia congenita A. Cl-channel B. Na-channel C. Ca-channel Part 3 (5-6) ( )5. Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) ( )6. Myoclonic epilepsy with ragged red fibers (MERRF) A ppint mutation B major deletion C point mutation D point mutation 1

2 Part 4 (7-8) ( )7. Phenylketonuria ( )8. Lesch-Nyhan syndrome A. Familial cerebral degenerative disease caused by a defect in branched-chain amino-acid metabolism B. Photosensitive dermatitis, intermittent cerebellar ataxia, mental disturbances and renal aminoaciduria C. An inborn error of metabolism, impairment in the hepatic hydroxylation of phenylalanine to tyrosine D. Sex-linked recessive disorder, oculocerebrorenal syndrome E. Hyperuricemia, mental retardation, choreoathetosis, and self-destructive biting of the lips and fingers. X-linked recessive trait. Part 5 (9-10) ( )9. Fabry disease ( )10. Gaucher disease A. Angiokeratoma corporis diffusum is an X-linked disorder affecting the skin, kidney, peripheral and autonomic nervous system. B. Normal in birth, severe vomiting, abdominal distention, diarrhea, poor weight gain, jaundice and unexplained fever in the first few weeks. C. genetically determined deficiency of the enzyme hexosaminidase D. Autosomal recessive sphingolipidosis in which glucocerebroside is stored. E. Lysosomal storage of the glycosphingolipid sphingomyelin. Part 6 (11-12) ( )11. Sturge-Weber Syndrome ( )12. Osler-Rendu-Weber Syndrome A. Port-wine nevus B. Axillary freckling C. Telangiectasias of the skin, mucous membranes and various internal organs D. Multiple retinal astrocytomas 2

3 Part 7 (13-14) Please match with the most appropriate activating procedure to help detecting the typical epileptiform discharges: ( )13. Juvenile myoclonic epilepsy ( )14. Infantile spasm A. Hyperventilation B. Intermittent photic stimulation C. Sleep Part 8 (15-16) ( )15. Phenytoin ( )16. Valproate A. Aplastic anemia and liver failure B. Cerebellar degeneration C. Tremor D. Renal stone E. Autoinduction Part 9 (17-18) ( )17. K-complex ( )18. slow eye movement A. stage I, II sleep B. stage III, IV sleep C. REM D. drowsy state 3

4 Part 10 (19-20) Common seizure patterns and their localizations ( )19. Auditory ( )20. Gustatory A. Heschl s gyri B. Prerolandic gyrus C. Frontal D. Temporal neocortex or amygdaloid-hippocampal comples E. Insula Part 11 (21-22) Types of facial pain related to the main lesion sites ( )21. postzoster neuralgia ( )22. Tolosa-Hunt syndrome A. 2 nd and 3 rd divisions of trigeminal N B. ophthalmic division of trigeminal N C. the pain is unilateral, behind or in front of ear, temple and face D. retro-orbital pain E. pain located at unilateral face, ear, jaws, teeth and upper neck F. the pain located at fronto-temporal and maxilla Part 12 (23-24) Severe cranial pain related to intracranial arterial dissection ( )23. Occipital pain ( )24. Occipital and supraorbital pain A. Basilar dissection B. Vertebral dissection C. Middle cerebral dissection 4

5 Part 13 (25-26) ( )25. Parinaud syndrome ( )26. Alien hand syndrome A. corpus callosum lesion B. pontine lesion C. midbrain lesion D. left temporal lobe lesion E. left parietal lobe lesion Part 14 (27-28) ( )27. Seesaw nystagmus ( )28. Slow relaxation of thenar muscles after handshake A. Midbrain lesion B. Colloid cyst of third ventricle C. Myotonic dystrophy D. Old upper motor neuron lesion E. Cerebellar hemisphere tumor F. Pontine lesion Part 15 (29-30) Match the cognition function and neuropsychological tests ( )29. wakefulness and attentional matrix ( )30. reasoning and abstraction A. Tower of London and Drawing a clock B. Wisconsin Card Sorting Test C. digital span D. Judgement of Line Orientation 5

6 Part 16 (31-32) Maternal exposures to the following agents may cause foetal abnormalities: ( )31. Craniofacial defects and finger nail hypoplasia ( )32. Congenital heart disease A. Phenytoin B. Carbamazepine C. Valproic acid D. Warfarin E. Herpex simple Part 17 (33-34) Heavy-mental poisoning ( )33. Colic, anemia, wrist drop, polyneuropathy and delirium ( )34. Tremor, ataxia of gait, confusion, blindness and sensory neuropathy A. Lead poisoing B. Aresic poisoning C. Mercury poisoning D. Manganese poisoning E. Thalium poisoning Part 18 (35-36) ( )35. Parkinsonian tremor ( )36. Essential tremor A. 16 Hz B. 4-6 Hz C. 6-8 Hz D. 3 Hz 6

7 Part 19 (37-38) Match the possible involuntary movements presented as drug adverse effect ( )37. Akathisia ( )38. Asterixia A. Estrogen B. Lithium C. Phenytoin D SSRI (serotonin selective reuptake inhibitor) Part 20 (39) Clinical correlation of basal ganglia lesions ( )39. Dystonia A. Subthalamic nucleus B. Caudate C. Putamen D. Substantia nigra Part 21 (40-41) ( )40. Myotonic dystrophy ( )41. Becker s muscular dystrophy A. Dystrophin protein B. DMPK protein C. Ryanodine receptor protein D. Myosin heavy chain protein 7

8 Part 22 (42-43) ( )42. Adult acid maltase deficiency ( )43. Pompe s disease A. Cramps and stiffness precipitated by brief high intensity exercise with onset 2 nd -3 rd decade B. Progressive proximal weakness plus respiratory insufficiency in adolescence or early adulthood and may mimic polymyositis or limb-girdle dystrophy C. Floppy baby, onset by about 3 months and death by about age 2 D. Recurrent attacks of rhabdomyolysis beginning in adolescence, precipitated by prolonged exercise, fasting and normal examination between attacks Part 23 (44-46) EMG findings and neuromuscular diseases ( )44. Lower motor neuron disorder ( )45. Myogenic lesion ( )46. Neuromuscular junction disorders A. Early recruitment B. Variable motor unit morphology C. Long-duration motor unit potential D. Slow firing rate Part 24 (47-48) Familial amyloid neuropathies (FAP) ( )47. Cranial neuropathy ( )48. Autonomic neuropathy A. FAP I B. FAP II C. FAP III D. FAP IV 8

9 Part 25 (49-52) ( )49. Normal pressure hydrocephalus ( )50. Congenital dislocation of hips ( )51. Charcot-Marie-Tooth disease ( )52. Vitamin B12 deficiency A. Apraxic gait B. Steppage gait C. Cerebellar gait D. Waddling gait E. Toppling gait F. Festinating gait G. Gait of sensory ataxia Part 26 (53-54) Extramedullary cranial nerve syndromes ( )53. Lateral wall of cavernus sinus ( )54. Jugular foramen A. II,III,IV, V,VI B. V,VI C. III,IV,V(1),VI D. III,IV,V(1,2),VI E. V,VII,VIII,IX F. IX,X,XI G. IX,X,XI,XII Part 27 (55-56) ( )55. Chvostek sign ( )56. Trousseau sign A. Lowering threshold of electrical excitability of the nerve B. Carpopedal spasm C. Evoking carpal spasm by inducing ischemia in the arm D. Contraction of the facial muscles on tapping the facial nerve in front of the ear 9

10 Part 28 (57-58) Chose the better match concerning pupil phenomenon ( )57. Small, irregular, unequal pupil and light-near dissociation ( )58. Degeneration of ciliary ganglia and post-ganglionic parasympthetic fibers A. Inverse Marcus Gunn phenomenon B. Marcus Gunn pupil C. Adie pupil D. Argyll Robertson pupil E. Hutchinson pupil Part 29 (59-60) The most common pathological location responsible for the following Nystagmus: ( )59. Ocular myoclonus. ( )60. Ocular bobbing A. Localized to cerebellum. B. Localized to pons. C. Localized to cervicalmedullary junction. D. Localized to midbrain. Part 30 (61-62) ( )61. Wernicke s aphasia ( )62. transcortical sensory aphasia A. Fluent with some articulatory defects, good comprehension and poor repetition B. Fluent, well articulated, poor comprehension and poor repetition C. Fluent, dysarthric, poor comprehension and fair repetition D. Fluent, scant and poor comprehension good repetition 10

11 Part 31 (63-64) ( )63. Cavernous sinus thrombosis ( )64. Superior sagittal sinus thrombosis A. The most frequently involved in aseptic cerebral venous thrombosis B. The most common form of septic cerebral venous thrombosis C. Associated with acute coma leading to death D. Most frequently associated with trigeminal nerve palsy Part 32 (65-66) ( )65. Fibromuscular dysplasia ( )66. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy A. Radiological appearance of involved vessels as irregular string of beads or a tubular narrowing B. Pulseless disease C. A mutation of the Notch 3 gene on chromosome 19 D. Occlusion of branches of the ophthalmic artery, resulting in blindness Part 33 (67-68) ( )67. Benedikt syndrome ( )68. Parinaud syndrome A. Fixed pupil, paralysis of upper gaze. B. Contralateral weakness and ipsilateral CN III palsy. C. Contralateral ataxia and tremor, ipsilateral CN III palsy. D. Contralateral ataxia, tremor and weakness and ipsilateral CN III palsy. 11

12 Part 34 (69-70) The symptoms and signs of ischemic stroke and structures involved ( )69. Anosognosia ( )70. Limb-kinetic apraxia A. Nondominant parietal lobe B. Supramarginal gyrus or inferior parietal lobe C. Posteromedial part of superior frontal gyrus (bilateral) D. Premotor or parietal cortical damage 12