Neurological Board Examination （I I） 2015 09 19

B-type: For each numbered item, select the best heading most closely associated with it. Each heading may be selected once, more than once, or not at all

Part 1 (1-4)

What is the most possible tumor presenting with the followings?

( ) 1. Foster Kennedy syndrome

( ) 2. Bitemporal hemianopia

( ) 3. Parinaud syndrome

( ) 4. Collier sign

A. Tumor in the Corpus Callosum

B. Pineal tumor

C. Large orbital tumor

D. Cerebellopontine angle tumor

E. Pituitary tumor

解答：( C) ( E) (B ) ( B) 題目之出處：Merritt’s Textbook of Neurology 12th Edition , Chapter 58. Page 373-374

Part 2 (5-8) Match the following syndromes and their symptoms: ( ) 5. Dejerine syndrome ( ) 6. Benedikt syndrome ( ) 7. Foville syndrome ( ) 8. Millard-Gubler syndrome

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A. Ipilateral facial nerve palsy, contralateral , gaze palsy to the Side of the lesion B. Contralateral hemiparesis, CN VI and CN VII palsies C. Contralateral hemiparesis, third nerve palsy, intension tremor, cerebellar D. Contralateral hemiparesis, dysarthria, loss of contralateral position and vibratory sensation, ipsilateral tongue paresis

解答：(D) (C) (A) (B)

題目之出處：Bradley's Neurology in Clinical Practice, 6th Edition, p278

Part 3 (9-12) Match the following vasculitic syndrome: ( ) 9. Wegener granulomatosis ( ) 10. Churg-Strauss syndrome ( ) 11. Polyarteritis nodosa ( ) 12. Henoch-Schonlen purpura

A. Necrotizing vasculitis of small-to-medium-sized vessels, with asthma and eosinophilia. B. Necrotizing inflammation of medium or small-sized arteries. No glomerulonephritis or vasculitis of arterioles, capillaries, venules. C. IgA-dominant immune deposits on small vessels. D. Necrotizing vasculitis of small-to-medium-sized vessels, with necrotizing glomerulonephritis. E. Necrotizing vasculitis with few or no immune deposits, affects small vessels. With necrotizing glomerulonephritis and involvement of pulmonary capillaries.

解答：(D), (A), (B), (C )

題目之出處： Merritt 12th, p. 325-326, table 50.1

Part 4 (13-16) Match the single-gene disorders associated with : ( ) 13. Fabry disease ( ) 14. Homocystinuria ( ) 15. CADASIL ( ) 16. Hereditary endotheliopathy with retinopathy, nephropathy and stroke

A. TREX 1 gene B. NOTCH3 gene C. α-galactosidase gene D. COL4A1 gene E. MTHFR gene

解答：( C ) ( E ) ( B ) ( A )

題目之出處：Merritt`s Neurology. 12th Edition, p.281

Part 5 (17-20) Please select the main mechanisms of action for following antiepileptic drugs (AEDs). ( ) 17. Vigabatrin ( ) 18. Lamotrigine ( ) 19. Gabapentin ( ) 20. Levetiracetam

A. Synaptic vesicle protein 2A B. Ca channel blocker C. GABA transaminase inhibitor D. GABA receptor agonist E. Na channel blocker

解答：( C ) ( E ) ( B ) ( A ) 題目之出處：台灣癲癇醫學會癲癇治療指引 2015

3 Part 6 (21-24) Clinical significance of electroencephalography finding： ( ) 21. Hypsarrhythmia ( ) 22. Periodic lateralized epileptiform discharges ( ) 23. Electrocerebral silence ( ) 24. Continuous focal slowing

A. Acute herpes simplex encephalitis B. West’s syndrome C. tumor D. Brain death E. Deep sleep

解答：( B ) ( A ) ( D ) ( C ) 題目之出處：Neurology in Clinical Practice, 5th Edition, P458-468

Part 7 (25-28) Match the characteristic clinical feature with the following epilepsy: ( ) 25. Juvenile myoclonic epilepsy ( ) 26. Lennox-Gastaut Syndrome ( ) 27. Rolandic epilepsy ( ) 28. West Syndrome

A. Lapse of consciousness as the core clinical manifestation B. Sudden flexor or extensor spasms involving the head, trunk and limbs simultaneously C. Atonic or astatic seizure D. Generalized tonic-clonic seizure upon awakening E. Clonic contractions of one side of the face, less often of one arm or leg

解答：(D) (C) (E) (B) 題目之出處： Merritt’s Neurology, 12th edition. P929; Adam p.307-313 Part 8 (29-32) Please match the motor-evoked potential (MEP) finding to the following choices: ( ) 29. Prolongation of the central motor conduction time ( ) 30. Increased MEP amplitude ( ) 31. Decrease MEP amplitude ( ) 32. Normal central motor conduction time

A. Single subthreshold TMS B. Paired-pulse TMS with an interstimulus interval of 2 milliseconds C. 20-Hz rTMS D. Parkinson’s disease E. Amyotrophic lateral sclerosis

解答：(E) (C) (B) (D)

題目之出處：Merritt’s Neurology 12th edition, Chapter 16, p.90-92

Part 9 (33-36) Please match the most possible symptom/sign with the neurological disease ( ) 33. Gowers sign ( ) 34. Glabellar sign ( ) 35. Hoover’s sign ( ) 36. Landry’s ascending paralysis

A. Guillian Barre syndrome B. Parkinson’s disease C. Psychogenic hemiparesis D. Polymyosis E. Neuromyelitis optica

解答：(D) (B) (C) (A) 題目之出處： Neurology in Clinical Practice, 6th edition, p. 265 ; Adam’s Principles of Neurology, 10th edition p.124

5 Part 10 (37-40) Match the corresponding spontaneous activities in the electromyographic study: ( ) 37. Myotonic discharges ( ) 38. Brief spike fibrillation potentials ( ) 39. Myokymic discharge ( ) 40. Complex repetitive discharges

解答：( C ) ( A ) ( D ) ( E )

Part 11 (41-44) Match the following nystagmus with most probable location of lesion: ( ) 41. Covergence-retraction nystagmus ( ) 42. Brun’s nystagmus ( ) 43. Upbeat nystagmus ( ) 44. Down beat nystagmus

A. Vestibulocerebellum B. Cerebellar vermis C. Cerebellopontine angle D. Rostral pretectum E. Pontine

解答: D, C, B, A 出處: The Neurology of Eye Movement, 4th edition (2006)

Part 12 (45-48) Please match the following symptoms and diseases. ( ) 45. , orofacial dyskinesia, feeding , self-mutilation ( ) 46. Blepharospasm with cranial ( ) 47. Risus sardonicus, dysarthria, dystonia, hyper-reflexia, dementia ( ) 48. Chorea, myoclonus, seizure, ataxia, dementia

A. Dentatorubro-pallidoluysian atrophy (DRPLA) B. Meige syndrome C. Huntington disease D. Neuroacanthocytosis E. Pantothenate kinase-associated neurodegenration

解答：(D) (B) (E ) (A)

題目之出處： Merritt’s 12th edition p.666-667, 670, 725, 742

Part 13 (49-52) Which gene accounts for the disease? ( ) 49. Paroxysmal non-kinesigenic dyskinesia ( ) 50. Paroxysmal kinesigenic dyskinesia ( ) 51. Dopa-responsive dystonia (Segawa) ( ) 52. Oppenheim dystonia

A. Myofibrillogenesis regulator 1 (MR-1) B. TorsinA (TOR1A) C. GTP cyclohydrolase I (GCH1) D. ε-Sarcoglycan (SGCE) E. proline-rich transmembrane protein 2 (PRRT2) 7

解答：( A ) ( E) (C ) (B ) 題目之出處：Merritt’s Neurology, 12th edition, Chap 120

Part 14 (53-56) Match the most suitable treatment for the following diseases? ( ) 53. Chorea of Huntington disease ( ) 54. Paroxysmal kinesigenic dyskinesia ( ) 55. Parkinson disease dementia ( ) 56. Young onset Parkinson’s disease

A. Chlorpromazine B. Tetrabenazine C. Carbamazepine D. Dopamine agonist E. Rivastigmine

解答：(B) ( C) (E) (D)

題目之出處：Merritt’s Neurology, 12th edition, Chap 116-122

Part 15 (57-60) The infusion rates of antiepileptic drugs (AED) during status epilepticus? ( ) 57. Phenytoin ( ) 58. Fosphenytoin ( ) 59. Valproic acid ( ) 60. Levetiracetam

A. < 50mg/min B. < 75mg/min C. < 100mg/min D. Drip >15 mins E. Drip >60 mins

解答：A, B, C, D 題目之出處：Lancet neurology Vol 14 June 2015

Part 16 (61-64) Patterns of breathing in patient with coma

( ) 61. Bilateral dysfunction of cerebral structures, usually deep in the hemisphere or diencephalon ( ) 62. Lower midbrain-upper pontine tegmentum ( ) 63. Lower pontine lesions ( ) 64. Dorsomedial part of the medulla

A. Apneustic breathing B. Central neurogenic hyperventilation C. Cheyne-Stokes respiration D. Biot breathing, also called “ataxia of breathing”

解答：C, B, A, D 題目之出處：Ａdam’s and Victor’s: Principles of neurology 10th , p. 374

Part 17 (65-68) Please match the following clinical symptoms with the diseases. ( ) 65. Sensory ataxia with impaired proprioceptive sensation, optic nerves atrophy, cerebral vasculitis ( ) 66. Aseptic meningitis, peripheral neuropathy, cranial nerves palsy ( ) 67. Trismus, risus sardonicus, and tonic spasms ( ) 68. Seizure, focal neurological deficit, movement disorders.

A. Whipple’s disease B. Japanese encephalitis C. Lyme disease D. Neurosyphilis 9 E. Tetanus

解答：(D) (C) (E) (B) 題目之出處：Merritt’s Neurology, 12th edition, chap. 26,30,32,34,36

Part 18 (69-72) Match the common systemic involvement for inherited myopathies: ( ) 69. Emery-Dreifuss muscular dystrophy ( ) 70. Myotonic dystrophy (DM1) ( ) 71. Mitochondrial myopathies ( ) 72. Duchenne muscular dystrophy

A. Ventricular arrhythmias B. Severe kyphoscoliosis C. Seizures D. Contrcatures of the elbows and Achilles tendons E. Hearing impairment

解答： (D), (A), (C), (B) 題目之出處： Myology, 3rd ed

Part 19 (73-76) Please match the following diseases or disorders with the corresponding antibodies. ( ) 73. Interstitial lung disease ( ) 74. Churg-Strauss syndrome ( ) 75. Multifocal motor neuropathy with conduction block ( ) 76. Stiff-man syndrome

A. Anti-GM1 B. Anti-Jo-1 C. Anti-MuSK D. ANCA E. Anti-GAD

解答：( B ) ( D ) ( A ) ( E )

題目之出處：Principles of Neurology 8th edition, chapter 46, p.1138 and p.1162, chapter 49, p.1207, and chapter 55, p.1279

Part 20 (77-80) Match the following inherited motor neuron disease with the associated gene: ( ) 77. Amyotrophic lateral sclerosis 1 ( ) 78. Amyotrophic lateral sclerosis 2 ( ) 79. FTD-PD-17 amyotrophy ( ) 80. Progressive muscular atrophy

A. Senataxin B. Dynactin C. SOD1 D. Tau E. Alsin

解答：(C) (E) (D) (B) 題目之出處卷：Merritt’s Neurology, 12th Ed p.804

Part 21 (81-84) Match the correct diagnosis with the following pathologic features: ( ) 81. The lesion begins in the median raphe and may involve all or part of the base of the . ( ) 82. Chronic demyelinating plaques are most frequently found in the periventricular white matter, optic nerves, brain stem, , and spinal cord. ( ) 83. Extensive demyelination and axonal damage and contain an inflammatory infiltrate consisting mainly of macrophages and B lymphocytes. ( ) 84. Necrosis of the medial zone of the corpus callosum.

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A. Multiple sclerosis B. Acute fulminant necrotizing encephalitis C. Central pontine myelinolysis D. Marchiafava-Bignami disease E. Neuromyelitis optica

解答：(C) (A) (E) (D) 題目之出處：Merritt's Neurology 12th edition, p. 903-926 考古

Part 22 (85-88) Match the side effect with disease modifying drugs in demylinating diseases: ( ) 85. Mitoxantrone ( ) 86. Natalizumab ( ) 87. β-interferons ( ) 88. Glatiramer

A. Progressive multifocal leukoencephalopathy B. Irreversible cardiotoxicity C. Flu-like symptoms D. Lipoatrophy E. Fatal hepatotoxicity

解答：(B) (A) (C) (D) 考古

Part 23 (89-92) Match the common side effects with the following migraine prophylactic agents

( ) 89. Propranolol ( ) 90. Valproic acid ( ) 91. Topiramate ( ) 92. Amitriptyline

A. QT prolongation B. Hair loss C. Urolithiasis D. Suicidality E. Impotance

解答：(E) (B) (C) (A) 題目之出處：Merritt’s Neurology, 11th edition, p. 955 題目屬性： Headache

Part 24 (93-96) Match one optimal treatment for each headache syndrome. ( ) 93. Chronic migraine ( ) 94. Hemicrania continua ( ) 95. Cluster headache ( ) 96. Hypnic headache

A. Caffeine B. Indomethacin C. Botulinum toxin injection D. 100% oxygen inhalation E. Carbamazepine

解答：(C) (B) (D) (A) 題目之出處： Merritt’s Neurology 12th Ed. Chap 155; Adams and Victor’s Principle of Neurology 10th Ed. Chapter 10

13 Part 25 (97-100) Each of these disorders tends to be marked pathologically by the abnormal accumulation in the brain of specific aggregated proteins. Please correlate them with the protein pathologies: ( ) 97. Dementia with Lewy bodies ( ) 98. Pick disease ( ) 99. ALS with dementia ( ) 100. Familial fatal insomnia

A. TDP-43 proteinopathy B. α-Synucleinopathy C. Tauopathy D. β-Amyloidopathy E. Prionopathy

解答：(B) (C) (A) (E) 題目之出處：Merritt’s Neurology, 12th edition, P 711.

Part 26 (101-104) Choose the related anatomic area for each of the following symptoms： ( ) 101. Dressing aparxia ( ) 102. ( ) 103. ( ) 104. Anton syndrome

A. Splenium of the corpus callosum B. Right C. Bilateral D. Right E. Left temporal lobe

解答：( D ) ( B ) ( A ) ( C ) 題目之出處：Merritt’s neurology, 11th edition, p8-13

Part 27 (105-108) Choose the related pathology for each of the following disease: ( ) 105. Corticobasal degeneration ( ) 106. Multiple system atrophy ( ) 107. Progressive supranuclear palsy ( ) 108. Frontotemporal dementia

A. Tufted astrocytes B. Pick’s bodes C. Astrocytic plaques D. Glial cytoplasmic inclusions E. Neurofibrillary tangles

解答：( C ) ( D ) ( A ) ( B ) 題目之出處：Principles of Neurology, 10e. Chap. 39

Part 28 (109-112) Physical characteristic for neurocutaneous syndromes: ( ) 109. Café-au-leit spots ( ) 110. Adenoma sebaceum ( ) 111. Shagreen patches ( ) 112. Facial nevus flammeus

A. Tuberous sclerosis B. Encephalotrigeminal angiomatosis C. Neurofibromatosis D. Incontinentia pigmenti E. Von Hipel-Lindau disease

解答：( C ) ( A ) ( A ) ( B )

15 Part 29 (113-116) The treatment of sleep disorders: ( ) 113. Modafinil ( ) 114. Ropinirole ( ) 115. Clonazepam ( ) 116. Clomipromine

A. Restless legs syndrome B. Sleep paralysis C. Narcolepsy D. Kleine-Levin syndrome E. REM sleep behavior disorder

解答：( C ) ( A ) ( E ) ( B ) 題目之出處：Adams and Victor’s Principles of Neurology, 10th edition, Chap.19, Sleep and its Abnormalities

Part 30 (117-120) Match the following test with autonomic function: ( ) 117. Cyclic deep breathing ( ) 118. BP response to the VM ( ) 119. Sympathetic skin response (SSR) ( ) 120. Schirmer test

A. Adrenergic B. Cardiovagal C. Sudomotor D. Salivary

解答：(B ) (A ) (C ) (D ) 題目之出處：Merritt’s textbook of Neurology 12th ed., Chap. 17, p93-95 考古