NBR 2 - Neuropathology Flashcards
1
Q
- Gambar:
A. choroid plexus papilloma
B. ependymoma
C. medulloblastoma
D. meningioma
E. pituitary adenoma
A
Jawab (C)
CNBR Fig. 3-85. Dense, hyperchromatic cells are radially arranged in Homer-Wright rosettes with central granulofibrillar material.
2
Q
- Gambar
A. choroid plexus papilloma
B. craniopharyngioma
C. hemangioblastoma
D. metastatic tumor
E. myxopapillary ependymoma
A
Jawab (E)
CNBR Fig. 3-71. Mucinous material accumulating about a hyalinalized vessel wall is characteristic.
3
Q
- Gambar:
A. choroid plexus papilloma
B. craniopharyngioma
C. hemangioblastoma
D. metastatic tumor
E. myxopapillary ependymoma
A
Jawab (B)
CNBR Fig. 3-102. Palisading epithelial cells with keratinization and calcification are prominent
4
Q
- Gambar:
A. choroid plexus papilloma
B. craniopharyngioma
C. hemangioblastoma
D. metastatic tumor
E. myxopapillary ependymoma
A
Jawab (C)
CNBR Fig. 3-100. Vacuolated “stromal” cells in a complex capillary network are seen.
5
Q
- Gambar:
A. butterfly glioma
B. carbon monoxide poisoning
C. fat emboli
D. lipoma
E. lipofuscin deposition
A
Jawab (D)
Ok Fig. 7-64A, p.257. Lipoma of the corpus callosum
6
Q
- Gambar:
A. carbon monoxide poisoning
B. fat emboli
C. Hallervorden-Spatz disease
D. miliary tuberculosis
E. Wilson’s disease
A
Jawab (A)
Nelson fig. 10-3, p. 178. Selective necrosis of the globus pallidus is noted.
7
Q
- Gambar:
A. ependymoma
B. GBM
C. medulloblastoma
D. meningioma
E. schwannoma
A
Jawab (B)
CNBR Fig. 3-59. Necrosis with pseudopalisading is well illustrated in this GBM.
8
Q
- Gambar:
The patient in this photograph is most likely to have
A. metastatic disease
B. NF – 1
C. NF – 2
D. tuberous sclerosis
E. von Hippel-Lindau disease
A
Jawab (D)
CNBR fig. 3-177. The adenoma sebaceum of tuberoussclerosis is seen.
9
Q
- Gambar:
A. Anaplastic astrocytoma
B. medulloblastoma
C. meningioma
D. metastatic tumor
E. oligodendriglioma
A
Jawab (C)
CNBR Fig. 3-93. Meningotheliomatous (syncytial) type of meningioma.
10
Q
- Gambar:
A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma
A
Jawab (C)
CNBR Fig. 3-94. Fibrous type of meningioma.
11
Q
- Gambar:
A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma
A
Jawab (D)
CNBR Fig. 3-149. Bundles of elongated Schwann’s cells with characteristic wavy nuclei in a loose mucinous or collagenous matrix are features of the neurofibroma.
12
Q
- Gambar:
A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma
A
Jawab (B)
CNBR Fig. 3-152. Malignant peripheral nerve sheath tumors are composed of spindle cells in fascicles with occasional mitoses and foci of necrosis
13
Q
- Gambar:
A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma
A
Jawab (E)
CNBR Fig. 3-145. Verocay bodies, palisading elongated nuclei encircling anuclear fibrillary material, are hallmarks of schwannomas.
14
Q
- Gambar:
This lesion is associated with the
A. filum terminale
B. kidney
C. notochord
D. pituitary
E. von Hippel-Lindau disease
A
Jawab (C)
CNBR Fig. 3-130. Chordomas feature “physaliferous” or “bubbly” cells Surrounding pools of mucin
15
Q
- Gambar:
A. Alzheimer’s disease
B. HSV-l
C. Huntington’s disease
D. Parkinson’s disease
E. Pick’s disease
A
Jawab (C)
Nelson Fig. 11-9, p. 230. Atrophy of the head of the caudate with “boxcar” ventricles is noted.
16
Q
- Gambar:
A. bacterial meningitis
B. candidiasis
C. neuritic plaques
D. neurofibrillary tangles
E. pick’s bodies
A
Jawab (C)
CNBR Fig. 3-189. Neuritic (“senile”) plaques (composed of degenerating nerve cell processes surrounding a central core of amyloid composed of ß/A4 protein) and neurofibrillary tangles are seen
17
Q
- Gambar:
A. acute disseminated encephalomyelitis
B. adrenoleukodystrophy
C. alexander’s disease
D. krabbe’s disease
E. metachromatic leukodystrophy
A
Jawab (C)
Nelson Fig. 13-41, p. 262. Numerous Rosenthal fibers (eosinophilic material in cell processes) in areas of astrocytosis are noted.
18
Q
- Gambar:
A. amyloid angiopathy
B. Duret’s hemorrhage
C. GBM
D. hypertensive hemorrhage
E. melanoma
A
Jawab (D)
Nelson Fig. 8-10a, p. 127. A massive basal ganglia hypertensive hemorrhage is noted.
19
Q
- Gambar:
A. central neuroblastoma
B. colloid cysts
C. GBM
D. hemangioblastoma
E. schwannoma
A
Jawab (A)
CNBR Fig. 3-91. A dense array of uniform undifferentiated cells with small blue nuclei and perinuclear halos is found in neurocytomas. The findings are similar to oligodendrogliomas, but central neurocytomas stain with synaptophysin and neuron-specific enolase (NSE).
20
Q
- Gambar:
A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration
A
Jawab (B)
CNBR Fig. 3-279. Posterior columns, spinocerebellar tracts, and corticospinal tracts are affected.
21
Q
- Gambar
A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration
A
Jawab (E)
Ok Fig. 6-12A, p. 195. There is a spongiform and gliotic appearance of the cord primarily affecting the posterior and lateral columns.
22
Q
- Gambar:
A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration
A
Jawab (D)
Ok Fig. 6-16B, p. 198. There is an irregular area of coagulation necrosis involving both gray and white matter.
23
Q
- Gambar:
A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration
A
Jawab (C)
CNBR Fig. 3-282. Well-defined plaques are seen.
24
Q
- Gambar
A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration
A
Jawab (A)
CNBR Fig. 3-276. The anterior horn and corticospinal tract are affected
25
144. Gambar:
A. gliomatosis cerebri
B. Huntington's disease
C. Krabbe's disease
D. multiple sclerosis
E. tuberous sclerosis
Jawab (E)
Nelson Fig. 20-10, p. 453. Cortical tubers are seen in the frontal and temporal lobes.
26
145. Gambar
A. choroid plexus papilloma
B. ependymoma
C. lymphoma
D. medulloblastoma
E. meningioma
Jawab (B)
CNBR Fig. 3-70. The histologic appearance of ependymomas is highly variable. A cellular variety with sheetlike growth of oval to polygonal cells arranged in a pseudovascular rosette is illustrated.
27
146. Gambar:
A. chordoma
B. dermoid
C. metastatic tumor
D. myxopapillary ependymoma
E. teratoma
Jawab (E)
Nelson Fig. 16-161, p. 365. Cartilage, mucin-producing epithelium, and immature spindle cell stroma are all part of this immature teratoma
28
147. Gambar:
A. advanced age and lobar hemorrhages
B. alcoholism and prone to falls
C. port-wine stain on the face
D. retinal angiomas
E. subungual fibromas
Jawab (C)
Ok fig. 7-72A, p. 264. Atrophy of the hemisphere and leptomeningeal venous angioma are present in this specimen with Sturge-Weber syndrome.
29
148. Gambar:
A. acoustic neuroma
B. anaplastic astrocytoma
C. medulloblastoma
D. melanoma
E. meningioma
Jawab (A)
CNBR Fig. 3-143. Dense Antoni A areas (with compact spindle cells) and looser Antoni B areas (with stellate cells) are typical of an acoustic neuroma (schwannoma).
30
149. Gambar
A. carbon monoxide poisoning
B. cerebral contusions
C. herpes encephalitis
D. meningeal carcinomatosis
E. metastatic melanomas
Jawab (D)
CNBR Fig. 3-137. Epitheloid cells with melanin inclusions are seen.
31
150. Gambar:
A. acoustic neuroma
B. anaplastic astrocytoma
C. medulloblastoma
D. melanoma
E. meningioma
Jawab (B)
CNBR Fig. 3-57. Cellular pleomorphism, hypercellularity, and mitotic activity characterize this anaplastic astrocytoma.
32
152. Gambar:
A. anaplastic astrocytoma
B. Infact
C. metachromatic leukodystrophy
D. multiple sclerosis
E. radiation necrosis
Jawab (C)
CNBR Fig. 3-188. Large confluent areas of dysmyelination with U-fiber sparing is typical of metachromatic leukodystrophy.
33
151 Gambar:
A. anaplastic astrocytoma
B. Infact
C. metachromatic leukodystrophy
D. multiple sclerosis
E. radiation necrosis
Jawab (D)
The periventricular plaques of multiple sclerosis are seen.
34
153. Gambar:
A. epidermoid
B. lipoma
C. metastatic tumor
D. multiple sclerosis
E. teratoma
Jawab (A)
Ok Fig. 7-62A, p. 255. A large cerebellopontine angle epidermoid with white flaky, keratinous debris is illustrated.
35
154. Gambar:
A. astrocytoma
B. lymphoma
C. melanoma
D. oligodendroglioma
E. pituitary adenoma
Jawab (B)
CNBR Fig. 3-139. A perivascular infiltration of cells with pleomorphic nuclear features characterizes this lymphoma.
36
155. Gambar
A. meningioma
B. neurofibroma
C. pilocystic astrocytoma
D. pleomorphic xanthoastrocytoma
E. schwannoma
Jawab (C)
CNBR Fig. 3-51. Compact fascicles of elongated cells and spongiform foci with stellate forms and microcystic change are noted.
37
156. Gambar:
A. aneurisma subarachnoid hemorrhage
B. bacterial meningitis
C. contusion
D. HSV – 1
E. subdural hematoma
Jawab (C)
Nelson Fig. 9.18A, p. 149. Extensive bilateral contrecoup contusions of the orbital surfaces and frontal poles are illustrated.
38
157. Gambar
A. amyloid angiopathy
B. arteriovenous malformation
C. capillary telangiectasia
D. embolism
E. venous angioma
Jawab (A)
CNBR Fig. 3-204A. The arterioles of the leptomeninges and superficial cortex are dilated, and amorphous material infiltrates the wall.
39
158. Gambar:
A. Alzheimer's disease
B. astrocytoma
C. Huntington's disease
D. Krabbe's disease
E. Pick's disease
Jawab (E)
Nelson Fig. 11.7, p. 228. Selective atrophy of the frontal and temporal lobes consistent with Pick's disease is noted.
40
159. Gambar:
A. Astrocytoma
B. GBM
C. hemangioblastoma
D. medulloblastoma
E. metastasis
Jawab (C)
Ok Fig. 7-48A, p. 245. The vascular mural nodule in the left cerebellar hemisphere and the associated cyst (midline) is consistent with a cerebellar hemangioblastoma.
41
160. Gambar:
A. Astrocytoma
B. GBM
C. neurofibroma
D. pituitary adenoma
E. schwannoma
Jawab (D)
CNBR Fig. 3-112. The normal acinar, heterogenous appearance of the pituitary is replaced by a diffuse sheet of polygonal cells.
42
161. Gambar:
A. Dejerine-Sottas disease
B. Krabbe's disease
C. metachromatic leukodystrophy
D. normal peripheral nerve
E. Charcot-Marie-Tooth disease
Jawab (D)
CNBR Fig. 3-308. A section from normal sural nerve is illustrated.
43
1. The organism most frequently identified in brain abscesses is
A. Bacteroides
B. Candida
C. Citrobacter
D. microaerophilic Streptococcus
E. Staphylococ cus
Jawab : D
Microaerophilic and anaerobic streptococci are the most frequently identified organisms in brain abscesses.
44
For questions 2 to 9, match the metal with the toxidty 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
Jawab : A
45
For questions 2 to 9, match the metal with the toxidty or description. Each response may be used once, more than once, or not at all.
A. arsenic
B. lead
C. mercury
D. manganese
3. psychological dysfunction ("mad as a hatter")
Jawab : C
46
For questions 2 to 9, match the metal with the toxidty or description. Each response may be used once, more than once, or not at all.
A. arsenic
B. lead
C. mercury
D. manganese
4. Parkinson's symptoms
Jawab : D
47
For questions 2 to 9, match the metal with the toxidty or description. Each response may be used once, more than once, or not at all.
A. arsenic
B. lead
C. mercury
D. manganese
5. red blood cell basophylic stippling
Jawab : C
48
For questions 2 to 9, match the metal with the toxidty or description. Each response may be used once, more than once, or not at all.
A. arsenic
B. lead
C. mercury
D. manganese
6. Brain levels increased by dimercaprol (BAL).
Jawab : C
49
For questions 2 to 9, match the metal with the toxidty or description. Each response may be used once, more than once, or not at all.
A. arsenic
B. lead
C. mercury
D. manganese
7. Symptoms improve with L-dopa.
Jawab : D
50
For questions 2 to 9, match the metal with the toxidty or description. Each response may be used once, more than once, or not at all.
A. arsenic
B. lead
C. mercury
D. manganese
8. increased urine coproporphyrin
Jawab : B
51
For questions 2 to 9, match the metal with the toxidty or description. Each response may be used once, more than once, or not at all.
A. arsenic
B. lead
C. mercury
D. manganese
9. Both penicillamine and BAL are used in treatment.
Jawab : B
52
For questions 10 to 14, match the structure with the description. Each response may be used once, more than once, or not at all.
A. neurofibrillary tangles
B. neuritic plaques
C. both
D. neither
10. intranuclear
Jawab : D
Neurofibrillary tangles and neuritic plaques are both intracytoplasmic;both contain paired helical filaments and are revealed with silver stains. The central core of the plaque is composed of p/A4, not a protein. The tangles are immunoreactive for z protein.
53
For questions 10 to 14, match the structure with the description. Each response may be used once, more than once, or not at all.
A. neurofibrillary tangles
B. neuritic plaques
C. both
D. neither
11. Core composed of Cl protein.
Jawab : D
Neurofibrillary tangles and neuritic plaques are both intracytoplasmic;both contain paired helical filaments and are revealed with silver stains. The central core of the plaque is composed of p/A4, not a protein. The tangles are immunoreactive for z protein.
54
For questions 10 to 14, match the structure with the description. Each response may be used once, more than once, or not at all.
A. neurofibrillary tangles
B. neuritic plaques
C. both
D. neither
12. contains paired helical filaments
Jawab : C
Neurofibrillary tangles and neuritic plaques are both intracytoplasmic;both contain paired helical filaments and are revealed with silver stains. The central core of the plaque is composed of p/A4, not a protein. The tangles are immunoreactive for z protein.
55
For questions 10 to 14, match the structure with the description. Each response may be used once, more than once, or not at all.
A. neurofibrillary tangles
B. neuritic plaques
C. both
D. neither
13. immunoreactive for t protein
Jawab : A
Neurofibrillary tangles and neuritic plaques are both intracytoplasmic;both contain paired helical filaments and are revealed with silver stains. The central core of the plaque is composed of p/A4, not a protein. The tangles are immunoreactive for z protein.
56
For questions 10 to 14, match the structure with the description. Each response may be used once, more than once, or not at all.
A. neurofibrillary tangles
B. neuritic plaques
C. both
D. neither
14. revealed with silver stains
Jawab : C
Neurofibrillary tangles and neuritic plaques are both intracytoplasmic;both contain paired helical filaments and are revealed with silver stains. The central core of the plaque is composed of p/A4, not a protein. The tangles are immunoreactive for z protein.
57
15. Most meningiomas express immunoreactivity for
A. cytokeratin
B. desmin
C. glial fibrillary acidic protein (GFAP)
D. S-100 protein
E. vimentin
Jawab : E
Vimentin and epithelial membrane antigen are expressed by the majority of meningiomas.
58
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
Jawab : D
Gangliogliomas are usually well circumscribed and can be partially cystic.
59
17. Which of the following is not associated with trisomy 13?
A. holoprosencephaly
B. hypertelorism
C. microcephaly
D. microphthalmia
E. polydactyly
Jawab : B
Hypotelorism, not hypertelorism, is associated with trisomy 13.
60
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
Jawab : A
61
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
19. Wernicke's encephalopathy
Jawab : A
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
62
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
20. Korsakoffs psychosis
Jawab : A
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
63
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
21. pellegra
Jawab : B
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
64
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
22. beriberi
Jawab : A
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
65
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
23. seen in rice eaters
Jawab : A
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
66
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
24. seen in corn eaters
Jawab : B
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
67
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
25. rickets
Jawab : E
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
68
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
26. pernicious anemia
Jawab : C
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
69
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
27. subacute combined degeneration
Jawab : C
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
70
For questions 19 to 28, match the vitamin with the description of its deficiency or toxicity. Each response may be used once, more than once, or not at all.
A. thiamine
B. niacin
C. vitamin B12
D. vitamin A
E. vitamin D
28. pseudotumor
Jawab : D
Diets heavy in corn lack tryptophan that is used to synthesize niacin. Diets heavy in refined rice are more likely to lack sufficient thiamine. Vitamin A toxicity may cause cerebral edema with a pseudotumor presentation. Pernicious anemia can lead to a B12 deficiency with megaloblastic anemia and subacute combined degeneration of the spinal cord.
71
29. Which of the following is true of lymphomas (non-Hodgkin's malignant lymphomas) of the central nervous system (eNS)?
A. All exhibit a diffuse histologic pattern.
B. Meningeal lesions are more common in primary lymphomas.
C. Most are ofT-ceil lineage.
D. Parenchymal lesions are more common in secondary lymphomas.
E. They are radioresistant.
Jawab : A
Meningeal infiltration is the most common lesion in secondary lymphomas,and parenchymal lesions are the most common lesion in primary lymphomas. Most are of B-cell lineage and are radiosensitive. Nodular lymphomas are not seen in the central nervous system (CNS); all show a diffuse histology.
72
30. Which ofthe following is not seen in Sturge-Weber syndrome?
A. cortical arteriovenous malformations
B. facial nevus
C. intracortical calcification
D. meningeal angioma
E. seizures
Jawab : A
Cortical arteriovenous malformations (AVMs) are not characteristic of the Sturge-Weber syndrome.
73
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.
Jawab : B
The demyelination, spongiosis, and gliosis seen in vitamin B12 deficiency are most common at lower cervical and thoracic levels.
74
32. Which of the following is associated with progressive multi focal 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
Jawab : B
Progressive multifocal leukoencephalopathyis caused by a papovavirus (notably the JC virus). Lesions occur mainly in the white matter and consist of foci of myelin and oligodendroglial cell loss with minimal inflammatory infiltrate. Hyperchromatic enlarged oligodendroglial nuclei are found at the margin of the lesions.
75
33. Which of the following is associated with von Hippel-Lindau disease?
I. hepatic cysts
II. hemangioblastoma ofthe 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
Jawab : E
Other associations are retinal hemangioblastomas, adrenal tumors, and polycythemia. The latter results from the production of a hematopoietic factor by the tumor.
76
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
Jawab : B
Schwannomas are charac- terized by a biphasic cellular pattern composed of compact spindle cells (Antoni A areas) and loosely arranged stellate cells (Antoni B areas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear fibrillar material. Neurofibromas incorporate the parent nerve and hence have axons in their midst. The plexiform type is considered pathognomonic for neurofibromatosistype 1.
77
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
35. Antoni B areas
Jawab : B
Schwannomas are charac- terized by a biphasic cellular pattern composed of compact spindle cells (Antoni A areas) and loosely arranged stellate cells (Antoni B areas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear fibrillar material. Neurofibromas incorporate the parent nerve and hence have axons in their midst. The plexiform type is considered pathognomonic for neurofibromatosistype 1.
78
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
36. Verocay bodies
Jawab : B
Schwannomas are charac- terized by a biphasic cellular pattern composed of compact spindle cells (Antoni A areas) and loosely arranged stellate cells (Antoni B areas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear fibrillar material. Neurofibromas incorporate the parent nerve and hence have axons in their midst. The plexiform type is considered pathognomonic for neurofibromatosistype 1.
79
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
37. Axons are present between tumor cells.
Jawab : A
Schwannomas are charac- terized by a biphasic cellular pattern composed of compact spindle cells (Antoni A areas) and loosely arranged stellate cells (Antoni B areas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear fibrillar material. Neurofibromas incorporate the parent nerve and hence have axons in their midst. The plexiform type is considered pathognomonic for neurofibromatosistype 1.
80
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
38. The plexiform type is strongly associated with neurofibromatosis type 1.
Jawab : A
Schwannomas are charac- terized by a biphasic cellular pattern composed of compact spindle cells (Antoni A areas) and loosely arranged stellate cells (Antoni B areas). Also seen are Verocay bodies, which result from the palisading of elongated nuclei alternating with anuclear fibrillar material. Neurofibromas incorporate the parent nerve and hence have axons in their midst. The plexiform type is considered pathognomonic for neurofibromatosistype 1.
81
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
Jawab : B
Hemorrhage is also common in melanoma, renal cell carcinoma, and lung carcinoma.
82
For questions 40 to 44, match the time period after a cerebral infarct with the histologic appearance. Each response may be used once, more than once, or not at all.
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
Jawab : C
83
For questions 40 to 44, match the time period after a cerebral infarct with the histologic appearance. Each response may be used once, more than once, or not at all.
A. 12 to 24 hours
B. days 1-2
C. days 5-7
D. days 10-20
E. more than 3 months
41. Fibrillary astrocytes present at the periphery of the lesion.
Jawab : E
84
For questions 40 to 44, match the time period after a cerebral infarct with the histologic appearance. Each response may be used once, more than once, or not at all.
A. 12 to 24 hours
B. days 1-2
C. days 5-7
D. days 10-20
E. more than 3 months
42. Gemistocytic astrocytes present at the periphery of the lesion.
Jawab : D
85
For questions 40 to 44, match the time period after a cerebral infarct with the histologic appearance. Each response may be used once, more than once, or not at all.
A. 12 to 24 hours
B. days 1-2
C. days 5-7
D. days 10-20
E. more than 3 months
43. polymorphonuclear infiltrate
Jawab : B
86
For questions 40 to 44, match the time period after a cerebral infarct with the histologic appearance. Each response may be used once, more than once, or not at all.
A. 12 to 24 hours
B. days 1-2
C. days 5-7
D. days 10-20
E. more than 3 months
44. Neuronal necrosis is first apparent.
Jawab : A
87
45. Hepatic failure is most closely associated with
A. endothelial proliferation
B. gliosis localized to the globus palIidus and hippocampus
C. gliosis localized to the white matter
D. Alzheimer's type II astrocytes
E. loss of oligodendroglial cells
Jawab : D
Acquired hepatocerebral degeneration is associated with gliosis with a predilection for the cortex. It tends to spare the hippocampus, globus pallidus, and deep folia of the cerebellar cortex. Widespread hyper- plasia of protoplasmic astrocytes is visible in the deep layers of the cerebral and cerebellar cortex and in deep nuclear structures.
88
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
Jawab : E
Vitamin A excess has not been associated with central pontine myelinolysis.
89
47. Rosenthal fibers are associated with
I. astroeytosis
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 ofthe above
Jawab : A
Rosenthal fibers, eosinophilic masses observed in astrocytic processes, are associated with pilocytic astrocytomas (neoplastic), and astrocytosis and Alexander's disease (nonneoplastic).
90
48. Which of the following is not typically seen in neurofibromatosis type 2?
A. acoustic neuromas
B. cafe au lait spots
C. cutaneous neurofibromatosis
D. Lisch nodules
E. plexiform neurofibromas
Jawab : D
Neurofibromas and cafe au lait spots occur less com- monly in neurofibromatosis type 2 (NF-2) than in NF-I. Bilateral acoustic neuromas are the hallmark of NF-2. Lisch nodules are rare in NF-2.
91
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
Jawab : A
92
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
Jawab : A
The motor nuclei of cranial nerves V, VII, IX, and XI1 as well as the motor\*cortex may be affected.
93
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
Jawab : C
Cavernous hemangiomas are composed of large. thin-walled vessels without interposed brain parenchyma.
94
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. micro tubules
B. oxidative phosphorylation
C. tanscription
D. translation
E. turnaround transport
52. diabetes
Jawab : E
95
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. micro tubules
B. oxidative phosphorylation
C. tanscription
D. translation
E. turnaround transport
53. vincristine nocnm
Jawab : A
96
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. micro tubules
B. oxidative phosphorylation
C. tanscription
D. translation
E. turnaround transport
54. mercury
Jawab : D
97
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. micro tubules
B. oxidative phosphorylation
C. tanscription
D. translation
E. turnaround transport
55. actinomycin D
Jawab : C
98
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. micro tubules
B. oxidative phosphorylation
C. tanscription
D. translation
E. turnaround transport
56. dinitrophenol
Jawab : B
99
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. micro tubules
B. oxidative phosphorylation
C. tanscription
D. translation
E. turnaround transport
57. vinblastine
Jawab : A
100
58. Catecholamine production can occur in which of the following tumors?
A. choriocarcinomas
B. glomus jugulare tumors
C. oligodendrogliomas
D. pineocytomas
E. pleomorphic xanthoastrocytomas
Jawab : B
Glomus jugulare tumors originate from foci of paraganglionic tis- sue around the jugular bulb. These invasive tumors contain neurosecretory granules similar to those in the carotid body. Some of them produce clinically detectable amounts of catecholamine.
101
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
Jawab : D
heviral inclusionsof herpessimplextype 1 (Cowdry type A) are dense, intranuclear, eosinophilic bodies found in neurons, astrocytes, and oligodendrocytes. They are more likely to befound early in the course of the disease.
102
60. High levels of alpha-fetoprotein are associated with
A. endodermal sinus tumors
B. choriocarcinomas
C. germinomas
D. pineoblastomas
E. teratomas
Jawab : A
High levels of human chorionic gonadotrophin are associated with choriocarcinomas, and high levels of alpha-fetoprotein are associated with endodermal sinus tumors (yolk sac tumors).
103
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
Jawab : B
104
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
Jawab : C
105
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
63. greatest tendency to hemorrhage
Jawab : B
106
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
64. Meningeal involvement is most common.
Jawab : D
107
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
65. least propensity to involve the brain
Jawab : E
108
For questions 66 to 69, match the mechanism of action to the disease. Each response may be used once, more than once, or not at all.
A. presynaptic inhibition at neuromuscular junction
B. inhibition of Renshaw cells
C. postsynaptic inhibition
66. botulism
Jawab : A
109
For questions 66 to 69, match the mechanism of action to the disease. Each response may be used once, more than once, or not at all.
A. presynaptic inhibition at neuromuscular junction
B. inhibition of Renshaw cells
C. postsynaptic inhibition
67. myasthenia gravis
Jawab : C
110
For questions 66 to 69, match the mechanism of action to the disease. Each response may be used once, more than once, or not at all.
A. presynaptic inhibition at neuromuscular junction
B. inhibition of Renshaw cells
C. postsynaptic inhibition
68. Eaton-Lambert syndrome
Jawab : A
111
For questions 66 to 69, match the mechanism of action to the disease. Each response may be used once, more than once, or not at all.
A. presynaptic inhibition at neuromuscular junction
B. inhibition of Renshaw cells
C. postsynaptic inhibition
69. tetanus
Jawab : B
112
70. Tuberous sclerosis is most closely associated with
A. acoustic neuromas
B. cortical calcification
C. giant -cell astrocytomas
D. optic gliomas
E. renal cysts
Jawab : C
113
71. High levels of human chorionic gonadotrophin are seen in
A. choriocarcinoma
B. embryonal carcinoma
C. endodermal sinus tumor
D. germinoma
E. teratoma
Jawab : A
114
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
Jawab : B
115
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. Sandhoffs disease
E. Tay-Sachs disease
73. sphingomyelinase deficiency
Jawab : C
116
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. Sandhoffs disease
E. Tay-Sachs disease
74. hexosaminidase A and B deficiency
Jawab : D
117
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. Sandhoffs disease
E. Tay-Sachs disease
75. glucocerebrosidase deficiency
Jawab : B
118
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. Sandhoffs disease
E. Tay-Sachs disease
76. Hexosaminidase A deficiency only
Jawab : E
119
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. Sandhoffs disease
E. Tay-Sachs disease
77. Alfa-galactosidase deficiency
Jawab : A
120
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. Sandhoffs disease
E. Tay-Sachs disease
78. abnormal accumulation of ceramide trihexosides
Jawab : A
121
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. Sandhoffs disease
E. Tay-Sachs disease
79. Tay-Sachs and this disorder are forms of the GM2 gangliosidases.
Jawab : D
122
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. Sandhoffs disease
E. Tay-Sachs disease
80. Supranuclear paresis of vertical gaze is highly characteristic.
Jawab : C
123
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. Sandhoffs disease
E. Tay-Sachs disease
81. Episodes of pain occur.
Jawab : A
124
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. Sandhoffs disease
E. Tay-Sachs disease
82. X-linked recessive
Jawab : A
125
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. Sandhoffs disease
E. Tay-Sachs disease
83. Cherry-red spots are found in virtually all patients with Sandhoffs and this disorder.
Jawab : E
126
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 q-L-iduronidase
Jawab : B
127
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)
85. characterized by severe skeletal deformities and ligamentous laxity
Jawab : C
128
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)
86. Heparan sulfate only is excreted in the urine.
Jawab : D
129
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)
87. deficiency of iduronate sulfatase; pebbling of the skin may occur; X-linked recessive
Jawab : A
130
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)
88. All forms of Morquio's and this disorder are characterized by normal intelligence.
Jawab : E
131
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
Jawab : D
132
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
90. deficiency of peroxisomes
Jawab : A
133
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
91. Rosenthal fibers are prominent
Jawab : B
134
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
92. Deficiency of aryl sulfatase
Jawab : E
135
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
93. X-linked recessive inheritance
Jawab : A
136
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
94. accumulation of small quantities of psychosine, a highly toxic compound
Jawab : D
137
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
95. accumulation of long-chain fatty acids
Jawab : A
138
96. Each ofthe following is characteristic of Wilson's disease except
A. Alzheimer's type II astrocytes
B. atrophy and brownish discoloration of the globus pallid us and putamen
C. autosomal dominant trait
D. decreased serum ceruloplasmin
E. decreased serum copper
Jawab : C
Wilson's disease is transmitted as an autosomal reces- sive trait.
139
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
Jawab : C
Both idiopathic Parkinson's disease and the Shy-Drager syndrome (a form of striatonigral degeneration) are characterized by loss of cells in the zona compacta of the substantia nigra, but in the latter disease is also found significant cell loss in the puta- men and the intermediolateral column. Lewy bodies are not found in the Shy-Drager syndrome. Patients with this syndrome suffer from parkinsonian symptoms and orthostatic hypotension.
140
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
98. loss of cells in the intermediolateral horn cells
Jawab : B
Both idiopathic Parkinson's disease and the Shy-Drager syndrome (a form of striatonigral degeneration) are characterized by loss of cells in the zona compacta of the substantia nigra, but in the latter disease is also found significant cell loss in the puta- men and the intermediolateral column. Lewy bodies are not found in the Shy-Drager syndrome. Patients with this syndrome suffer from parkinsonian symptoms and orthostatic hypotension.
141
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
99. Lewy bodies present
Jawab : A
Both idiopathic Parkinson's disease and the Shy-Drager syndrome (a form of striatonigral degeneration) are characterized by loss of cells in the zona compacta of the substantia nigra, but in the latter disease is also found significant cell loss in the puta- men and the intermediolateral column. Lewy bodies are not found in the Shy-Drager syndrome. Patients with this syndrome suffer from parkinsonian symptoms and orthostatic hypotension.
142
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
100. prominent loss of neurons in the putamen
Jawab : B
Both idiopathic Parkinson's disease and the Shy-Drager syndrome (a form of striatonigral degeneration) are characterized by loss of cells in the zona compacta of the substantia nigra, but in the latter disease is also found significant cell loss in the puta- men and the intermediolateral column. Lewy bodies are not found in the Shy-Drager syndrome. Patients with this syndrome suffer from parkinsonian symptoms and orthostatic hypotension.
143
101. The most common neurologic complication of acquired immunodeficiency syndrome (AIDS) is
A. dementia
B. inflammatory polymyositis
C. lymphoma
D. myelopathy
E. toxoplasmosis
Jawab : A
Dementia, characterized by cognitive dysfunction, behavioral disturbance, and motor impairment, occurs in one-third to two-thirds of patients with AIDS. Myelopathy occurs in less than lo%, inflammatory polymyositis in 20%,toxoplasmosis in lo%,and lymphoma in 5%of AIDS patients.
144
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
Jawab : B
The triad of adenoma sebaceum (actually angiofibro- mas), epilepsy, and mental retardation characterizes tuberous sclerosis. Although benign tumors (angiomyolipomas) of the kidney and other organs are seen, renal cell carcinomas are not. The other lesions listed are associated.
145
103. Each of the following is seen in neurofibromatosis type 1 except
A. axillary freckling
B. cafe au lait macules
C. neurofibromas of the iris
D. optic gliomas
E. sphenoid dysplasia
Jawab : C
Hamartomas (not neurofibromas) of the iris (Lisch nod- ules) are szen in NF-I.
146
104. Each of the following is true of amyloid angiopathy except
A. amyloid beta-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.
Jawab : C
Cerebral amyloid angiopathy is confined to intracranial arteries and arterioles in the leptomeninges and superficial cortex.
147
105. Characteristic pathologic findings in Guillain-Barre 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
Jawab : B
The CSF in Guillain-Barre syndrome is under normal pressure, is acellular in 90%of patients, and demonstrates an increased protein level that peaks at 4 to 6 weeks after onset. Responses D and E are characteristic findings.
148
106.
A. rarely multiple
B. associated with immunosuppression in older men
C. associated with immunosuppression in younger men
D. resistant to steroids
Jawab : C
his is lymphoma with diffuse perivascular lympho- cytic infiltration into the Virchow-Robin space around a blood vessel. Such lymphomas are often multiple, respond initially to steroids (but invariably recur), and are associated with immunosuppression in younger men. They also occur in immunocompetent males over 60 years.
149
107.
A. Hirano bodies
B. inclusion bodies of subacute sclerosing panencephalitis (SSPE)
C. Lewy bodies
D. Pick's bodies
E. rabies
Jawab : B
150
108.
A. Hirano bodies
B. inclusion bodies of SSPE
C. Lewybodies
D. Pick's bodies
E. rabies
Jawab : B
The inclusion bodies of HSV (dense, eosinophilic, and sur- rounded by a clear halo) and SSPE are intranuclear. The inclusion bodies seen in the other responses are intra cytoplasmic. Smaller eosinophilic intra cytoplasmic inclusions may also be seen in SSPE.
151
109.
A. inclusion bodies of herpes simplex virus type 1 (HSV-l)
B. Lewy bodies
C. Pick's bodies
D. rabies
E. inclusion bodies of SSPE
Jawab : D
Pick's bodies (rounded intracytoplasmic masses) are visible in the pyramidal neurons of the hippocampus.
152
110.
A. inclusion bodies of HSV-l
B. Lewybodies
C. Pick's bodies
D. rabies
E. inclusion bodies of SSPE
Jawab : B
Lewy bodies (neuronal intracytoplasmic inclusions with and eosinophilic core surrounded by a clear halo) are seen in the neurons of the substantia nigra.
153
111.
A. Ganglioglioma
B. Hepatic encephalophaty
C. HSV-1
D. Parkinson’s disease
E. normal cortex
Jawab : B
Alzheimer's type 1astrocytes (large vesicular nuclei and little visible cytoplasm) are present. These are found in hepatic encephalopathy and Wilson's disease.
154
112.
A. Fibrillary astrocytoma
B. gemistrocytic astrocytoma
C. glioblastoma multiforme
D. hemangioblastoma
E. oligodendroglioma
Jawab : B
The cells of this variant of astrocytoma have prominent eosinophilic cytoplasm, short processes, and eccentric nuclei.
155
113.
A. aneurysmal subarachnoid hemorrhage
B. bacterial meningitis
C. contusion
D. HSV-l
E. subdural hematoma
Jawab : D
The hemorrhagic appearance of the medial temporal lobe is characteristic of HSV-I.
156
114.
A. AIDS encephalopathy
B. giant-cell glioblastoma multiforme (GBM)
C. hemangioblastoma
D. Creutzfeldt-Jakob disease
E. progressive multifocalleukoencephalopathy
Jawab : E
Demyelination and oligodendroglial cell loss are seen. Residual oligodendroglial nuclei are large and bizarre.
157
115.
A. AIDS encephalopathy
B. giant-cell GBM
C. hemangioblastoma
D. Creutzfeldt-jakob disease
E. progressive multifocal leukoencephaltopathy
Jawab : D
Numerous vacuoles give a spongiform appearance.
158
116.
A. metastatic disease
B. NF – 1
C. NF – 2
D. tuberous scelorosis
E. von hippel-Lindau disease
Jawab : B
The iris hamartomas (Lisch nodules) of NF-I are seen.
159
117.
A. AIDS encephalopathy
B. giant-cell GBM
C. hemangioblastoma
D. Creutzfeldt-Jakob disease
E. progressive multifocalleukoencephalopathy
Jawab : A
Perivascular foci of inflammation with prominent multinu- cleated giant cells are typical.
160
118.
A. anaplastic astrocytoma
B. ependymoma
C. ganglioglioma
D. meningioma
E. oligodendroglioma
Jawab : C
The finding of abnormal ganglion cells (including binucleate forms) is key to the diagnosis of ganglioglioma.
161
119.
A. anaplastic astrocytoma
B. meningioma
C. normal pituitary gland
D. oligodendroglioma
E. pituitary adenoma
Jawab : D
CNBR Fig. 3-68. A "chicken wire" vascular pattern and a monotonous nuclear array are seen.
