Neurological Board Examination (I I)

Neurological Board Examination (I I) 2006 09 16 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 all Part 1 (1-4) ( ) 1. Skin Biopsy ( ) 2. In multifocal motor neuropathy ( ) 3. In Guillain Barré syndrome ( ) 4. Myotonic Dystrophy A. anti-gm1a, GM1b, GD1a and GalNAc-GD1a antibodies are found in patients with acute motor axonal neuropathy (AMAN) B. subclinical polyneuropathy is common and may represent one of the manifestations of the multisystem involvement. C. A New Window on Small-fiber Sensory Neuropathy D. IgM anti-gm1 antibodies (which cross react with GD1b and GA1) are detectable in around 50% of cases From Acta Neurologica Taiwanica Answer: C, D, A, B Part 2 (5-8) ( ) 5. multiple system atrophy ( ) 6. idiopathic Parkinson disease ( ) 7. progressive supranuclear palsy ( ) 8. corticobasal degeneration A. ocular abnormalities preceding gaze palsy B. primary progressive aphasia C. cerebellar dysfunction D. unilateral onset and persistent asymmetry affecting the side of symptom Onset Answer: C, D, A, B (from Parkinson s disease & movement disorders, 4 th ed, Jankovic J.J. & Tolosa E., p 159, p189, p171, & p176) 1

Part 3 (9-12) ( ) 9. Seizure disorder ( ) 10. Nightmares ( ) 11. REM sleep behavior disorder ( ) 12. Sleep walking (somnambulism) A. REM B. NREM C. Wake D. NREM>wake>REM Answer: 1. D, 2. A, 3. A, 4.B (Sleep medicine From textbook of Merritt s neurology, 11 th ed, 2005) Part 4 (13-16) ( ) 13. Hypokalemic periodic paralysis ( ) 14. Hyperkalemic periodic paralysis ( ) 15. Myotonia congenita, Becker type ( ) 16. Malignant hyperthermia A. chloride channel disorder B. calcium channel disorder C. ryanodine receptor disorder D. sodium channel disorder Answer 1. (B), 2. (D), 3. (A), 4. (C) (Principles of Neurology 8 th edition, Adams and Victors) Part 5 (17-20) ( ) 17. Temporal arteritis ( ) 18. Takayasu disease ( ) 19. Wegener granulomatosis ( ) 20. Fibromuscular dysplasia A. mononeuropathy multiplex B. Pulseless disease C. Transverse constrictions, giving the appearance of an irregular string of beads or a tubular narrowing in angiography D. Occlusion of branches of the ophthalmic artery, resulting in blindness Answer 1. D, 2. B, 3. A, 4. C (From Principles of Neurology, 7 th ed.) 2

Part 6 (21-24) ( ) 21. Wilson s disease ( ) 22. Myoclonus dystonia ( ) 23. Corticobasal degeneration ( ).24. Multiple system atrophy A. Autonomic dysfunction B. Apraxia C. Liver cirrhosis D. ε sarcoglycan gene mutation (From Neurology in Clinical Practice 4th edition p2140,2142,2158,2159) Answer: C, D, B, A Part 7 (25-26) Brainstem syndromes ( ) 25. Millard-Gubler syndrome ( ) 26. Parinaud syndrome A. Paralysis of soft palate and vocal cod plus ipsilateral tongue paralysis and contralateral hemianesthesia B. Facial and abducens palsy and contralateral hemiplegia C. Oculomotor palsy with contralateral cerebellar ataxia D. Oculomotor palsy with contralateral hemiplegia E. Paralysis of upward gaze and accommodation F. Ipsilateral V, IX, X, XI palsy Ans: B, E (Stroke. From Adams and Victor s Principle of Neurology 8 th ed. p 681 Table 34-3) Part 8 (27-31) Match the seizure pattern and its localization ( ) 27. Jacksonian seizure ( ) 28. Contraversive seizure ( ) 29. Vertiginous ( ) 30. Olfactory hallucination ( ) 31. Gustatory hallucination A. Prerolandic gyrus B. Insula C. Superior temporal lobe D. Mesial temporal lobe E. Supplementary motor cortex Ans: A, E, C, D, B (Epilepsy. Adams 8 th ed p275 table 16-2) 3

Part 9 (32-34) Match the characteristically possible image finding to the following headache syndrome: ( ) 32. Cough induced headache ( ) 33. Idiopathic thunderclap headache ( ) 34. Hypertensive encephalopathy A. Arnold-Chiari malformation B. Posterior leucoenphalopathy C. Intracranial segmental vasospasm Ans: A, C, B Part 10 (35-36) Choose the specific neurotransmitter in the following area ( ) 35. Raphe neucli ( ) 36. Locus cerulus A. NE B. Acetylcholine C. Serotonin D. Glutamate E. GABA Ans: C, A (Neuroscience, Adams. Principles of Neurology 8 th ed. p59 fig4.4; p336) Part 11 (37-40) ( ) 37. Amantadine ( ) 38. Pergolide ( ) 39. Tolcapone ( ) 40. Pramipexole and Ropinirole A. acute hepatic necrosis B. sleep attack C. reduce dyskinesia in advanced PD D. development of valvular heart disease Answer: C, D, A, B (From Clinical Neurology, 6 th ed., MJ Aminoff) 4

Part 12 (41-44) The abnormality on blink reflex examination, when stimulate right side ( ) 41. Severe lesion of the right interneuron to ipsilateral facial neucleus ( ) 42. Severe lesion of right trigeminal nerve ( ) 43. Severe lesion of right facial nerve ( ) 44. Severe lesion of right spinal neucleus A. Diminished right R2 B. Diminished left R2 C. Diminished bilateral R2 D. Diminished right R1 and R2 E. Diminished all R1 and R2 response Ans: A, E, D, C (Acta Neurologica Taiwanica & annual meeting s paper. Acta Neurologica Taiwanica, 2006;15:2-12) Part 13 (45-48) The following symptoms and neurocutaneous syndrome ( ) 45. Progressive ataxia with hypersensitivity to ionizing radiation ( ) 46. Child with hemiparesis, focal and generalized seizures, and an erythematous skin lesion ( ) 47. Café-au-lait lesions with optic glioma ( ) 48. Paradoxical embolism with stroke and higher risk of meningitis or brain abscess A. Nuerofibromatois 1 B. Neurofibromatosis 2 C Tuberous sclerosis complex D. Osler-Weber-Rendu syndrome E. Sturge- Weber syndrome F. Ataxia telangiectasia Ans: F, E, A, D (Degenerative & genetic disease. NICP 4 th ed 1874-1885) 5

Part 14 (49-50) Link each of the following spontaneous muscle activities to the appropriate definition ( ) 49. Fibrillation ( ) 50. Complex repetitive discharge A. Regularly occurring spikes firing at 0.5 to 10 Hz with a sound similar to rain on a tin roof and initial positive deflection B. There is a spontaneous involuntary discharge of an individual motor unit C. Spikes are waxing and waning in both amplitudes and frequencies D. It occurred from depolarization of a single muscle fiber, followed by emphatic spread to adjacent denervated fibers Answer : A, D Part 15 (51-52) ( ) 51. Sydenham chorea ( ) 52. Neuroacanthocytosis A. Tardive dyskinesia B. Autoimmune disorder C. Pseudopurposeful movement D. High serum creatine kinase Answer: B, D (From Merrit 11 th version P 805,806, 807, 810, 815) Part 16 (53-56) Link the following diseases /syndromes to the correct inheritance pattern: ( ) 53. Neurofibromatosis type1 ( ) 54. Sturge-weber-demitri syndrome ( ) 55. Incontinentia pigmenti ( ) 56. Ataxia telangiectasis A. Autosomal dominant B. Most sporadic inheritance C. X-linked dominant D. Autosomal recessive Answer. A, B, C, D (From Merritt s 11 rd version P713,718,722,786) 6

Part 17 (57-60) ( ) 57. Rabies encephalitis ( ) 58. Herpes encephalitis ( ) 59. Progressive multi-focal leukodystrophy ( ) 60. CMV encephalitis A. Negri bodies B. Cowdry type A C. Oligodendrocyte with inclusion D. Owl s eye cells Answer: A, B, C, D (From Merritt s 11 rd version p188,191,201,196) Part 18 (61-64) Monogenic epileptic disorders with channelopathies ( ) 61. Juvenile myoclonic epilepsy ( ) 62. Benign familial neonatal convulsion ( ) 63. Episodic ataxia type 2 with spike-wave seizures ( ) 64. Benign infantile epilepsy A. Sodium channels B. Potassium channels C. Ligand-gated channels D. Calcium channels Ans : C, A, D, B (Epilepsy. Principles of Neurology, 8 th edition, Adams and Victor s) 7