Chapter 5 Neuropathology 1. The organism most frequently identified in brain abscesses is A. Bacteroides B. Candida C. Citrobacter D. microaerophilic Streptococcus E. Staphylococcus For questions 2 to 9, match the metal with the toxicity or description. Each response may be used once, more than once, or not at all. A. arsenic B. lead C. mercury D. manganese 2. Mees’ transverse white lines on fingernails 3. psychological dysfunction (“mad as a hatter”) 4. Parkinson’s symptoms 5. red blood cell basophylic stippling 6. Brain levels increased by dimercaprol (BAL). 7. Symptoms improve with L-dopa. 8. increased urine coproporphyrin 9. Both penicillamine and BAL are used in treatment. A. neurofibrillary tangles B. neuritic plaques C. both D. neither 10. intranuclear 11. Core composed of α protein. 12. contains paired helical filaments 13. immunoreactive for τ protein 14. revealed with silver stains 15. Most meningiomas express immunoreactivity for A. cytokeratin B. desmin C. glial fibrillary acidic protein (GFAP) D. S-100 protein E. vimentin 16. Each of the following is true of ganglioglioms except that A. The astrocytes are GFAP positive. B. The ganglion cells are synaptophysin positive. C. They contain neuropeptides. D. They are usually diffusely infiltrative. E. They are most common in the temporal lobes. 17. Which of the following is not associated with trisomy 13? A. holoprosencephaly B. hypertelorism C. microcephaly D. microphthalmia E. polydactyly 18. Which of the following is not characteristic of ependymomas? A. blepharoplasts in the basal cytoplasm B. intermediate filaments that are immunohistochemically identical to glial filaments of astrocytes C. perivascular pseudorosettes D. surface microvilli E. true rosette formation A. thiamine B. niacin C. vitamin B12 D. vitamin A E. vitamin D 19. Wernicke’s encephalopathy 20. Korsakoff’s psychosis 21. pellegra 22. beriberi 23. seen in rice eaters 24. seen in corn eaters 25. rickets 26. pernicious anemia 27. subacute combined degeneration 28. pseudotumor 29. Which of the following is true of lymphomas (non-Hodgkin’s malignant lymphomas) of the central nervous system (CNS)? A. All exhibit a diffuse histologic pattern. B. Meningeal lesions are more common in primary lymphomas. C. Most are of T-cell lineage. D. Parenchymal lesions are more common in secondary lymphomas. E. They are radioresistant. 30. Which of the following is not seen in Sturge-Weber syndrome? A. cortical arteriovenous malformations B. facial nevus C. intracortical calcification D. meningeal angioma E. seizures 31. Each of the following is true of the cord pathology in pernicious anemia except A. Demyelination occurs. B. Lumbar levels are most severely affected. C. Lesions may occur in the medulla. D. Vacuolar distention of myelin sheaths occur. E. Wallerian degeneration occurs. 32. Which of the following is associated with progressive multifocal encephalopathy? A. bacterial infection B. demyelination C. increased numbers of oligodendroglial cells D. intense inflammatory infiltrate E. shrunken oligodendroglial nuclei at the periphery of the lesion 33. Which of the following is associated with von Hippel-Lindau disease? I. hepatic cysts II. hemangioblastoma of the spinal cord III. renal cysts IV. renal cell carcinoma A. I, II, III B. I, III C. II, IV D. IV E. all of the above For questions 34 to 38, match the tumor with the description. Each response may be used once, more than once, or not at all. A. neurofibroma B. schwannoma C. both D. neither 34. Antoni A areas 35. Antoni B areas 36. Verocay bodies 37. Axons are present between tumor cells. 38. The plexiform type is strongly associated with neurofibromatosis type 1. 39. Which one of the following cerebral metastases has the greatest tendency to hemorrhage? A. breast B. choriocarcinoma C. gastrointestinal (GI) tract D. ovarian E. prostate A. 12 to 24 hours B. days 1–2 C. days 5–7 D. days 10–20 E. more than 3 months 40. lipid-laden macrophages first appear 41. Fibrillary astrocytes present at the periphery of the lesion. 42. Gemistocytic astrocytes present at the periphery of the lesion. 43. polymorphonuclear infiltrate 44. Neuronal necrosis is first apparent. 45. Hepatic failure is most closely associated with A. endothelial proliferation B. gliosis localized to the globus pallidus and hippocampus C. gliosis localized to the white matter D. Alzheimer’s type II astrocytes E. loss of oligodendroglial cells 46. Each of the following has been associated with central pontine myelinolysis except A. alcoholism B. severe burns C. rapid correction of hyponatremia D. serum hyperosmolarity E. vitamin A excess 47. Rosenthal fibers are associated with I. astrocytosis II. Alexander’s disease III. pilocytic astrocytoma IV. Pick’s disease A. I, II, III B. I, III C. II, IV D. IV E. all of the above 48. Which of the following is not typically seen in neurofibromatosis type 2? A. acoustic neuromas B. café au lait spots C. cutaneous neurofibromatosis D. Lisch nodules E. plexiform neurofibromas 49. Which of the following is not associated with hepatic encephalopathy? A. thiamine deficiency B. asterixis C. Alzheimer’s type II astrocytes D. increased serum ammonia 50. In amyotrophic lateral sclerosis, the cranial nerve nucleus that typically does not exhibit cell loss is A. III B. V C. VII D. IX E. XII 51. Which of the following vascular malformations have no intervening brain parenchyma between blood vessels? A. arteriovenous malformations B. capillary telangiectasias C. cavernous hemangiomas D. cryptic arteriovenous malformations E. venous angiomas For questions 52 to 57, match the sites of damage in the axonal transport apparatus with the toxin. Each response may be used once, more than once, or not at all. A. microtubules B. oxidative phosphorylation C. tanscription D. translation E. turnaround transport 52. diabetes 53. vincristine 54. mercury 55. actinomycin D 56. dinitrophenol 57. vinblastine 58. Catecholamine production can occur in which of the following tumors? A. choriocarcinomas B. glomus jugulare tumors C. oligodendrogliomas D. pineocytomas E. pleomorphic xanthoastrocytomas 59. The viral inclusions seen in herpes simplex encephalitis are A. basophilic B. called Cowdry type B bodies C. found in neurons only D. intranuclear E. only evident several weeks after the infection 60. High levels of alpha-fetoprotein are associated with A. endodermal sinus tumors B. choriocarcinomas C. germinomas D. pineoblastomas E. teratomas 61. The most common sites of hypertensive hemorrhage, in decreasing order of frequency, are A. lobar, putamen, cerebellum, thalamus, pons B. putamen, lobar, thalamus, cerebellum, pons C. putamen, thalamus, pons, lobar, cerebellum D. thalamus, cerebellum, lobar, putamen, pons E. thalamus, lobar, putamen, cerebellum, pons For questions 62 to 65, match the source of the metastatic brain lesion to the description. Each response may be used once, more than once, or not at all. A. breast B. choriocarcinoma C. lung D. lymphoma E. prostate 62. most common 63. greatest tendency to hemorrhage 64. Meningeal involvement is most common. 65. least propensity to involve the brain A. presynaptic inhibition at neuromuscular junction B. inhibition of Renshaw cells C. postsynaptic inhibition 66. botulism 67. myasthenia gravis 68. Eaton-Lambert syndrome 69. tetanus 70. Tuberous sclerosis is most closely associated with A. acoustic neuromas B. cortical calcification C. giant-cell astrocytomas D. optic gliomas E. renal cysts 71. High levels of human chorionic gonadotrophin are seen in A. choriocarcinoma B. embryonal carcinoma C. endodermal sinus tumor D. germinoma E. teratoma 72. Cushing’s syndrome is most often associated with a(n) A. acidophilic pituitary adenoma B. basophilic pituitary adenoma C. chromophobic pituitary adenoma D. ectopic source of adrenocorticotropic hormone (ACTH) E. nonfunctioning pituitary adenoma For questions 73 to 83, match the sphingolipidosis with the description. Each response may be used once, more than once, or not at all. A. Fabry’s disease B. Gaucher’s disease C. Niemann-Pick disease D. Sandhoff’s disease E. Tay-Sachs disease 73. sphingomyelinase deficiency 74. hexosaminidase A and B deficiency 75. glucocerebrosidase deficiency 76. Hexosaminidase A deficiency only 77. α-galactosidase deficiency 78. abnormal accumulation of ceramide trihexosides 79. Tay-Sachs and this disorder are forms of the GM2 gangliosidases. 80. Supranuclear paresis of vertical gaze is highly characteristic. 81. Episodes of pain occur. 82. X-linked recessive 83. Cherry-red spots are found in virtually all patients with Sandhoff’s and this disorder. For questions 84 to 88, match the mucopolysaccharidosis (MPS) with the description. Each response may be used once, more than once, or not at all. A. Hunter’s syndrome (MPS II) B. Hurler’s syndrome (MPS I H) C. Morquio’s syndrome (MPS IV) D. Sanfilippo’s syndrome (MPS III) E. Scheie’s syndrome (MPS I S) 84. deficiency of α-L-iduronidase 85. characterized by severe skeletal deformities and ligamentous laxity 86. Heparan sulfate only is excreted in the urine. 87. deficiency of iduronate sulfatase; pebbling of the skin may occur; X-linked recessive 88. All forms of Morquio’s and this disorder are characterized by normal intelligence. For questions 89 to 95, match the leukodystrophy with the description. Each response may be used once, more than once, or not at all. A. adrenoleukodystrophy B. Alexander’s disease C. Canavan’s disease D. Krabbe’s disease E. metachromatic leukodystrophy 89. Deficiency of galactocerebrosidase 90. deficiency of peroxisomes 91. Rosenthal fibers are prominent. 92. Deficiency of arylsulfatase 93. X-linked recessive inheritance 94. accumulation of small quantities of psychosine, a highly toxic compound 95. accumulation of long-chain fatty acids 96. Each of the following is characteristic of Wilson’s disease except A. Alzheimer’s type II astrocytes B. atrophy and brownish discoloration of the globus pallidus and putamen C. autosomal dominant trait D. decreased serum ceruloplasmin E. decreased serum copper For questions 97 to 100, match the description with the disease or syndrome. A. idiopathic Parkinson’s disease B. Shy-Drager syndrome C. both D. neither 97. loss of cells in the zona compacta of the substantia nigra 98. loss of cells in the intermediolateral horn cells 99. Lewy bodies present 100. prominent loss of neurons in the putamen 101. The most common neurologic complication of acquired immunodeficiency syndrome (AIDS) is A. dementia B. inflammatory polymyositis C. lymphoma D. myelopathy E. toxoplasmosis 102. Each of the following lesions is characteristic of tuberous sclerosis except A. adenoma sebaceum B. renal cell carcinoma C. rhabdomyomas of the heart D. subependymal giant-cell astrocytes E. subungual fibromas 103. Each of the following is seen in neurofibromatosis type 1 except A. axillary freckling B. café au lait macules C. neurofibromas of the iris D. optic gliomas E. sphenoid dysplasia 104. Each of the following is true of amyloid angiopathy except A. amyloid β protein is the major protein seen. B. Aneurysmal dilations are seen in involved vessels. C. It occurs primarily in vessels of deep nuclear structures of the brain. D. It occurs primarily in patients over 70 years of age. E. A yellow-green dichromism is seen under polarized light when the amyloid is stained with Congo red. 105. Characteristic pathologic findings in Guillain-Barré syndrome include each of the following except A. increased cerebrospinal fluid (CSF) protein at 5 weeks after onset of illness B. lymphocytic pleocytosis in 90% of patients C. normal CSF pressures D. perivascular lymphocytic and inflammatory cell infiltrate E. perivenule and segmental demyelination For questions 106 to 161, match the figure with the most appropriate response. 106.
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