| 1. The first step in screening for acute intermittent porphyria is to order a A. blood protoporphyrin assay B. genetic screen C. urine porphobilinogen (*) D. urine uroporphyrin E. therapeutic trial of phenobarbital 2. Patients with porphyria cutanea tarda are LEAST LIKELY to exhibit A. blisters on the backs of the hands B. coexisting iron overload and/or chronic viral hepatitis C. diminished delta-amino levulinic acid levels (*) D. extra hair on the face E. fluorescent pink urine 3. A "cotton wool patch" in the retina is the result of A. glaucoma B. hemorrhage C. ischemia (*) D. scar contraction E. toxoplasmosis 4. The classic cause of ophthalmia neonatorum was A. congenital syphilis B. excess oxygen in the preemie nursery C. gonorrhea (*) D. muscle imbalance E. rubella 5. The typical patient with periventricular leukomalacia is: A. a child with a mutation in a myelin gene B. a child with ataxia-telangiectasia C. a newborn with hypoxia around the time of birth (*) D. a patient at any age receiving chemotherapy E. an older person with atherosclerosis of the large arteries 6. In the current system (Daumas-Duport) of grading astrocytomas, which does NOT contribute toward a higher grade? A. bizarre nuclei B. presence of gemistocytes (*) C. presence of mitotic figures D. presence of necrosis E. proliferation of endothelium 7. All are known Alzheimer genes EXCEPT A. amyloid precursor protein B. apoprotein E C. presenilin 1 D. presenilin 2 E. synuclein (*) 8. Patients presenting with true gliosarcomas, with both glial and mesenchymal differentiation, typically have a history of A. ataxia-telangiectasia B. neurosurgery C. radiation exposure (*) D. Sturge-Weber E. tuberous sclerosis 9. A juvenile pilocytic astrocytoma is most likely to arise in the A. cerebellum (*) B. dorsal root ganglia C. frontal lobes D. retina E. spinal cord itself 10. The microscopic hallmark of diffuse axonal injury is A. Alzheimer type I glia B. Alzheimer type II glia C. astrocytic plaques D. Pick bodies E. "retraction ball" spheroids (*) 11. The one really treatable hereditary cerebellar degeneration results from a lack of the transfer protein which handles A. carnitine B. divalent cations C. vitamin B1 (thiamine) D. vitamin B12 E. vitamin E (*) 12. The "Charcot-Marie-Tooth" family of peripheral neuropathies show primarily A. abnormal eye movements B. atrophy of the lower legs (*) C. pain in the distal extremities D. pain in the proximal extremities E. weakness of proximal muscles 13. TWO KODACHROMES. What's your best diagnosis? A. central pontine myelinolysis B. idiopathic parkinsonism (*) C. multiple sclerosis D. old infarct E. post-encephalitic parkinsonism 14. TWO KODACHROMES. What are these lesions? A. coup contusions B. epidural hematomas C. hemorrhagic infarcts D. intracerebral hematomas E. subdural hematomas (*) 15. ONE KODACHROME. What's your best diagnosis? A. glioma of the cord B. multiple sclerosis C. neurofibroma D. syringomyelia (*) E. tabes dorsalis 16. TWO KODACHROMES. What is the diagnosis? A. congenital rubella B. neurofibromatosis (*) C. Sturge-Weber D. trisomy 13 E. tuberous sclerosis 17. ONE KODACHROME. What is this? A. astrocytoma, grade I B. cryptococcosis C. HIV encephalitis (*) D. lymphoma E. medulloblastoma 18. ONE KODACHROME. What is the diagnosis? A. Arnold-Chiari (*) B. diffuse axonal injury C. Pick's disease D. pontine glioma E. tuberous sclerosis 19. ONE KODACHROME. What is this brainstem lesion? A. central pontine myelinolysis B. Duret hemorrhage (*) C. multiple sclerosis D. pilocytic astrocytoma E. ruptured berry aneurysm 20. FOUR KODACHROMES. What is the diagnosis? A. brain abscess B. cerebral contusion C. glioblastoma (*) D. medulloblastoma E. tuberculosis 21. ONE KODACHROME. Look closely. The lesions in the basal ganglia are most likely due to A. Huntington's B. hypertensive microvascular disease (*) C. leukodystrophy D. kernicterus E. thiamine deficiency 22. ONE KODACHROME. What is the diagnosis? A. abscess B. lymphoma C. meningococcal infection D. recent hypoxic injury (*) E. tuberculosis 23. THREE KODACHROME. What is the diagnosis? A. abscess B. chordoma C. cryptococcus infection D. herpes simplex E. meningioma (*) 24. ONE KODACHROME. Look closely at this section of cerebellum. What is the diagnosis? A. alcoholism B. glioblastoma C. medulloblastoma D. rabies (*) E. Tay-Sachs 25. TWO KODACHROMES. What is the diagnosis? A. Alzheimer's B. adrenoleukodystrophy or something similar C. astrocytoma D. carbon monoxide effect E. multiple sclerosis (*) 26. TWO KODACHROMES. What is the diagnosis? A. abscess (*) B. glioblastoma C. medulloblastoma D. oligodendroglioma E. tuberculosis 27. ONE KODACHROME. Myelin stain. What's your diagnosis? A. amyotrophic lateral sclerosis (*) B. multiple sclerosis C. old poliomyelitis D. tabes dorsalis E. Werdnig-Hoffman 28. ONE KODACHROME. What is the diagnosis? Patient with lifelong handicap. A. holoprosencephaly B. lissencephaly (*) C. polymicrogyria D. tuberous sclerosis E. ulegyria 29. ONE KODACHROME. What is the diagnosis? Patient with lifelong handicap. A. congenital CMV B. old intraventricular hemorrhage (prematurity) C. perinatal ischemic injury (*) D. polymicrogyria E. porencephaly / schizencephaly 30. ONE KODACHROME. What is the infectious agent? A. aspergillus B. herpes simplex (*) C. meningococcus D. rabies E. tuberculosis 31. ONE KODACHROME. What is the diagnosis? A. bacterial meningitis B. cysticercosis C. epidural hematoma D. hemorrhagic infarct (*) E. subdural hematoma 32. ONE KODACHROME. Your diagnosis? A. bacterial meningitis B. chordoma C. contrecoup contusions (*) D. glioma of some sort E. tabes dorsalis 33. TWO KODACHROMES. What is the diagnosis? A. abscess B. ependymoma (*) C. medulloblastoma D. multiple sclerosis E. tuberculosis 34. ONE KODACHROME. What's the eye lesion? A. glaucoma B. optic nerve atrophy C. papilledema (*) D. retinal detachment E. Tay-Sachs 35. ONE KODACHROME. What do you see? A. arhinencephaly B. herpes encephalitis C. tonsillar herniation D. tuberculosis meningitis E. uncal herniation (*) 36. ONE KODACHROME. Myelin stain of the spinal cord. This is most suggestive of A. amyotrophic lateral sclerosis B. multiple sclerosis C. pernicious anemia (*) D. syringomyelia E. trauma 37. TWO KODACHROME. What is the most likely cause of this brainstem pathology? A. chordoma B. hemorrhage due to herniation C. oligodendroglioma D. syphilis E. tuberculosis (*) 38. THREE KODACHROMES. What's your best diagnosis? A. Alzheimer's disease (*) B. glioblastoma C. Huntington's chorea D. neurosyphilis E. no pathology 39. ONE KODACHROME. Look closely and think. A. Dandy-Walker (*) B. medulloblastoma C. multiple sclerosis D. superior vermal atrophy from alcohol abuse E. tonsillar herniation 40. ONE KODACHROME. What's the most likely diagnosis? A. Alzheimer's disease B. congenital hydrocephalus C. herpes simplex encephalitis D. Huntington's chorea (*) E. Parkinsonism 41. THREE KODACHROMES. What are these lesions? A. astrocytoma B. chronic subdural hematomas C. medulloblastoma D. old infarcts (*) E. tuberculosis 42. THREE KODACHROMES. What's your most likely diagnosis? A. Alzheimer's disease B. bacterial meningitis (*) C. herpes encephalitis D. neurosyphilis E. toxoplasmosis 43. ONE KODACHROME. Periodic acid - Schiff stain. What's your diagnosis? A. adenocarcinoma B. cryptococcosis (*) C. medulloblastoma D. metastatic carcinoma E. mucormycosis 44. ONE KODACHROME. What is this? A. cryptococcus B. multiple sclerosis C. old ischemic injury (*) D. toxoplasmosis E. tuberculosis 45. ONE KODACHROME. Give your best diagnosis. A. chordoma B. epidural hematoma (*) C. meningioma D. subarachnoid hemorrhage E. syphilitic pachymeningitis 46. ONE KODACHROME. Look carefully and think. What's this? A. arteriovenous malformation (*) B. cysticercosis C. epidural hematoma D. oligodendroglioma E. subdural hematoma 47. ONE KODACHROME. What's your diagnosis? A. Arnold-Chiari B. Dandy-Walker C. congenital CMV D. diffuse hypoxic injury E. tuberous sclerosis (*) 48. ONE KODACHROME. What is the problem in the optic fundus? A. atheroemboli B. glaucoma C. hypertensive microvascular disease (*) D. senile macular degeneration E. shaken baby syndrome BONUS ITEMS 49. TWO KODACHROMES. Your best diagnosis: [Pick's / frontotemporal atrophy] 50. TWO KODACHROMES. What's the name of this lesion? [encephalocele] 51. TWO KODACHROMES. What's the diagnosis? Be specific. [medulloblastoma] 52. TWO KODACHROMES. Your best diagnosis, please. [prion disease / spongiform encephalopathy / CJD / etc.] 53. TWO KODACHROMES. What's the diagnosis? [multiple sclerosis] 54. TWO KODACHROMES. What's the eye lesion? [retinoblastoma] 55. ONE KODACHROME. What is this lesion? [berry aneurysm] 56. What bacterium is the usual cause of both perichondritis of the pinna and "malignant external otitis" of the canal? [pseudomonas] 57. Tinnitus that pulsates with the heartbeat strongly suggests what benign tumor? [glomus jugulare tumor; accept any hemangioma] 58. What's a cholesteatoma? Show that you really know the answer. [some evidence that you know it's an epidermoid cyst of the middle ear] 59. "Buphthalmos" is a picturesque word for a dread condition. What does it mean? Just the right answer, please. ["cow eye" / swollen globe] 60. Why does the macula of the eye appear as a "red cherry" in Tay-Sach's storage disease? [contrast with pale, lipid-laden nerve layer] 61. What would you see at autopsy of someone dying of sudden impact syndrome without other pathology? [marked cerebral edema] 62. Where in the brain do most hemangioblastomas arise? [cerebellum] 63. A neuropathologist finds many neurofibrillary tangles and a damaged septum pellucidum. While waiting for the clinical history, the neuropathologist would suspect: [boxer] 64. As the words are usually used, how is "cerebritis" different from "encephalitis"? [cerebritis implies bacteria] 65. What would you expect to see on microscopic examination of a patient dying of West Nile Encephalitis? [lymphocytes around vessels and necrosis; I'll settle for lymphocytes] 66. What do we mean by a "watershed infarct" of the brain? [between arterial distributions] 67. At autopsy, why does edema fluid drip from the cut surfaces of the brains of people dying from lead poisoning, but not of people dying of Reye's syndrome? If you know this one, you're a neuropathology star. [vasogenic vs. cytotoxic edema] 68. Although metastatic carcinoma to the brain is common, we never see it in the brain's lymphatics. Why not? [the brain has no lymphatics] 69. What illness which commonly follows CMV or campylobacter infection is caused by an autoantibody against the tetanus/botulism binding site? [Guillain-Barr or synonym] 70. What brain lesion is unusually common in people with autosomal dominant polycystic kidney disease? [berry aneurysms] 71. Neurofibrillary tangles located primarily in the brainstem and basal ganglia suggest a diagnosis of: [progressive supranuclear palsy] 72. What is the name of the supposedly-neurotoxic eukaryote which overgrew the Chesapeake bay recently? [Pfeisteria piscocida; credit for any recognizable spelling] NAME:_ 48 points maximum UHS Pathology Nervous System 2003-2004 INSTRUCTIONS: You know the routine. Members of the first group need to leave all-together, without stragglers, when time is called. IF MEDICAL SCHOOL WERE EASY, YOUR DEGREE WOULD BE WORTHLESS! 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