NBR 2 - Neuropathology

Question 1

120. Gambar:

A. choroid plexus papilloma
B. ependymoma
C. medulloblastoma
D. meningioma
E. pituitary adenoma

Answer 1

Jawab (C)

CNBR Fig. 3-85. Dense, hyperchromatic cells are radially arranged in Homer-Wright rosettes with central granulofibrillar material.

Question 2

121. Gambar

A. choroid plexus papilloma
B. craniopharyngioma
C. hemangioblastoma
D. metastatic tumor
E. myxopapillary ependymoma

Answer 2

Jawab (E)

CNBR Fig. 3-71. Mucinous material accumulating about a hyalinalized vessel wall is characteristic.

Question 3

122. Gambar:

A. choroid plexus papilloma
B. craniopharyngioma
C. hemangioblastoma
D. metastatic tumor
E. myxopapillary ependymoma

Answer 3

Jawab (B)

CNBR Fig. 3-102. Palisading epithelial cells with keratinization and calcification are prominent

Question 4

123. Gambar:

A. choroid plexus papilloma
B. craniopharyngioma
C. hemangioblastoma
D. metastatic tumor
E. myxopapillary ependymoma

Answer 4

Jawab (C)

CNBR Fig. 3-100. Vacuolated “stromal” cells in a complex capillary network are seen.

Question 5

124. Gambar:

A. butterfly glioma
B. carbon monoxide poisoning
C. fat emboli
D. lipoma
E. lipofuscin deposition

Answer 5

Jawab (D)

Ok Fig. 7-64A, p.257. Lipoma of the corpus callosum

Question 6

125. Gambar:

A. carbon monoxide poisoning
B. fat emboli
C. Hallervorden-Spatz disease
D. miliary tuberculosis
E. Wilson’s disease

Answer 6

Jawab (A)

Nelson fig. 10-3, p. 178. Selective necrosis of the globus pallidus is noted.

Question 7

126. Gambar:

A. ependymoma
B. GBM
C. medulloblastoma
D. meningioma
E. schwannoma

Answer 7

Jawab (B)

CNBR Fig. 3-59. Necrosis with pseudopalisading is well illustrated in this GBM.

Question 8

127. 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

Answer 8

Jawab (D)

CNBR fig. 3-177. The adenoma sebaceum of tuberoussclerosis is seen.

Question 9

128. Gambar:

A. Anaplastic astrocytoma
B. medulloblastoma
C. meningioma
D. metastatic tumor
E. oligodendriglioma

Answer 9

Jawab (C)

CNBR Fig. 3-93. Meningotheliomatous (syncytial) type of meningioma.

Question 10

129. Gambar:

A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma

Answer 10

Jawab (C)

CNBR Fig. 3-94. Fibrous type of meningioma.

Question 11

130. Gambar:

A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma

Answer 11

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.

Question 12

131. Gambar:

A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma

Answer 12

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

Question 13

132. Gambar:

A. GBM
B. malignant peripheral nerve sheath tumor
C. meningioma
D. neurofibroma
E. schwannoma

Answer 13

Jawab (E)

CNBR Fig. 3-145. Verocay bodies, palisading elongated nuclei encircling anuclear fibrillary material, are hallmarks of schwannomas.

Question 14

133. Gambar:

This lesion is associated with the

A. filum terminale
B. kidney
C. notochord
D. pituitary
E. von Hippel-Lindau disease

Answer 14

Jawab (C)

CNBR Fig. 3-130. Chordomas feature “physaliferous” or “bubbly” cells Surrounding pools of mucin

Question 15

134. Gambar:

A. Alzheimer’s disease
B. HSV-l
C. Huntington’s disease
D. Parkinson’s disease
E. Pick’s disease

Answer 15

Jawab (C)

Nelson Fig. 11-9, p. 230. Atrophy of the head of the caudate with “boxcar” ventricles is noted.

Question 16

135. Gambar:

A. bacterial meningitis
B. candidiasis
C. neuritic plaques
D. neurofibrillary tangles
E. pick’s bodies

Answer 16

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

Question 17

136. Gambar:

A. acute disseminated encephalomyelitis
B. adrenoleukodystrophy
C. alexander’s disease
D. krabbe’s disease
E. metachromatic leukodystrophy

Answer 17

Jawab (C)

Nelson Fig. 13-41, p. 262. Numerous Rosenthal fibers (eosinophilic material in cell processes) in areas of astrocytosis are noted.

Question 18

137. Gambar:

A. amyloid angiopathy
B. Duret’s hemorrhage
C. GBM
D. hypertensive hemorrhage
E. melanoma

Answer 18

Jawab (D)

Nelson Fig. 8-10a, p. 127. A massive basal ganglia hypertensive hemorrhage is noted.

Question 19

138. Gambar:

A. central neuroblastoma
B. colloid cysts
C. GBM
D. hemangioblastoma
E. schwannoma

Answer 19

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).

Question 20

139. Gambar:

A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration

Answer 20

Jawab (B)

CNBR Fig. 3-279. Posterior columns, spinocerebellar tracts, and corticospinal tracts are affected.

Question 21

140. Gambar

A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration

Answer 21

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.

Question 22

141. Gambar:

A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration

Answer 22

Jawab (D)

Ok Fig. 6-16B, p. 198. There is an irregular area of coagulation necrosis involving both gray and white matter.

Question 23

142. Gambar:

A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration

Answer 23

Jawab (C)

CNBR Fig. 3-282. Well-defined plaques are seen.

Question 24

143. Gambar

A. amyotrophic lateral sclerosis
B. Friedreich’s ataxia
C. multiple sclerosis
D. radiation myelopathy
E. subacute combined degeneration

Answer 24

Jawab (A)

CNBR Fig. 3-276. The anterior horn and corticospinal tract are affected

Question 25

144. Gambar:

A. gliomatosis cerebri
B. Huntington’s disease
C. Krabbe’s disease
D. multiple sclerosis
E. tuberous sclerosis

Answer 25

Jawab (E)

Nelson Fig. 20-10, p. 453. Cortical tubers are seen in the frontal and temporal lobes.

Question 26

145. Gambar

A. choroid plexus papilloma
B. ependymoma
C. lymphoma
D. medulloblastoma
E. meningioma

Answer 26

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.

Question 27

146. Gambar:

A. chordoma
B. dermoid
C. metastatic tumor
D. myxopapillary ependymoma
E. teratoma

Answer 27

Jawab (E)

Nelson Fig. 16-161, p. 365. Cartilage, mucin-producing epithelium, and immature spindle cell stroma are all part of this immature teratoma

Question 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

Answer 28

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.

Question 29

148. Gambar:

A. acoustic neuroma
B. anaplastic astrocytoma
C. medulloblastoma
D. melanoma
E. meningioma

Answer 29

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).

Question 30

149. Gambar

A. carbon monoxide poisoning
B. cerebral contusions
C. herpes encephalitis
D. meningeal carcinomatosis
E. metastatic melanomas

Answer 30

Jawab (D)

CNBR Fig. 3-137. Epitheloid cells with melanin inclusions are seen.

Question 31

150. Gambar:

A. acoustic neuroma
B. anaplastic astrocytoma
C. medulloblastoma
D. melanoma
E. meningioma

Answer 31

Jawab (B)

CNBR Fig. 3-57. Cellular pleomorphism, hypercellularity, and mitotic activity characterize this anaplastic astrocytoma.

Question 32

152. Gambar:

A. anaplastic astrocytoma
B. Infact
C. metachromatic leukodystrophy
D. multiple sclerosis
E. radiation necrosis

Answer 32

Jawab (C)

CNBR Fig. 3-188. Large confluent areas of dysmyelination with U-fiber sparing is typical of metachromatic leukodystrophy.

Question 33

151 Gambar:

A. anaplastic astrocytoma
B. Infact
C. metachromatic leukodystrophy
D. multiple sclerosis
E. radiation necrosis

Answer 33

Jawab (D)

The periventricular plaques of multiple sclerosis are seen.

Question 34

153. Gambar:

A. epidermoid
B. lipoma
C. metastatic tumor
D. multiple sclerosis
E. teratoma

Answer 34

Jawab (A)

Ok Fig. 7-62A, p. 255. A large cerebellopontine angle epidermoid with white flaky, keratinous debris is illustrated.

Question 35

154. Gambar:

A. astrocytoma
B. lymphoma
C. melanoma
D. oligodendroglioma
E. pituitary adenoma

Answer 35

Jawab (B)

CNBR Fig. 3-139. A perivascular infiltration of cells with pleomorphic nuclear features characterizes this lymphoma.

Question 36

155. Gambar

A. meningioma
B. neurofibroma
C. pilocystic astrocytoma
D. pleomorphic xanthoastrocytoma
E. schwannoma

Answer 36

Jawab (C)

CNBR Fig. 3-51. Compact fascicles of elongated cells and spongiform foci with stellate forms and microcystic change are noted.

Question 37

156. Gambar:

A. aneurisma subarachnoid hemorrhage
B. bacterial meningitis
C. contusion
D. HSV – 1
E. subdural hematoma

Answer 37

Jawab (C)

Nelson Fig. 9.18A, p. 149. Extensive bilateral contrecoup contusions of the orbital surfaces and frontal poles are illustrated.

Question 38

157. Gambar

A. amyloid angiopathy
B. arteriovenous malformation
C. capillary telangiectasia
D. embolism
E. venous angioma

Answer 38

Jawab (A)

CNBR Fig. 3-204A. The arterioles of the leptomeninges and superficial cortex are dilated, and amorphous material infiltrates the wall.

Question 39

158. Gambar:

A. Alzheimer’s disease
B. astrocytoma
C. Huntington’s disease
D. Krabbe’s disease
E. Pick’s disease

Answer 39

Jawab (E)

Nelson Fig. 11.7, p. 228. Selective atrophy of the frontal and temporal lobes consistent with Pick’s disease is noted.

Question 40

159. Gambar:

A. Astrocytoma
B. GBM
C. hemangioblastoma
D. medulloblastoma
E. metastasis

Answer 40

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.

Question 41

160. Gambar:

A. Astrocytoma
B. GBM
C. neurofibroma
D. pituitary adenoma
E. schwannoma

Answer 41

Jawab (D)

CNBR Fig. 3-112. The normal acinar, heterogenous appearance of the pituitary is replaced by a diffuse sheet of polygonal cells.

Question 42

161. Gambar:

A. Dejerine-Sottas disease
B. Krabbe’s disease
C. metachromatic leukodystrophy
D. normal peripheral nerve
E. Charcot-Marie-Tooth disease

Answer 42

Jawab (D)

CNBR Fig. 3-308. A section from normal sural nerve is illustrated.

Question 43

1. The organism most frequently identified in brain abscesses is
   A. Bacteroides
   B. Candida
   C. Citrobacter
   D. microaerophilic Streptococcus
   E. Staphylococ cus

Answer 43

Jawab : D

Microaerophilic and anaerobic streptococci are the most frequently identified organisms in brain abscesses.

Question 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

Answer 44

Jawab : A

Question 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”)

Answer 45

Jawab : C

Question 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

Answer 46

Jawab : D

Question 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

Answer 47

Jawab : C

Question 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).

Answer 48

Jawab : C

Question 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.

Answer 49

Jawab : D

Question 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

Answer 50

Jawab : B

Question 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.

Answer 51

Jawab : B

Question 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

Answer 52

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.

Question 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.

Answer 53

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.

Question 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

Answer 54

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.

Question 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

Answer 55

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.

Question 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

Answer 56

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.

Question 57

15. Most meningiomas express immunoreactivity for
    A. cytokeratin
    B. desmin
    C. glial fibrillary acidic protein (GFAP)
    D. S-100 protein
    E. vimentin

Answer 57

Jawab : E

Vimentin and epithelial membrane antigen are expressed by the majority of meningiomas.

Question 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

Answer 58

Jawab : D
Gangliogliomas are usually well circumscribed and can be partially cystic.

Question 59

17. Which of the following is not associated with trisomy 13?
    A. holoprosencephaly
    B. hypertelorism
    C. microcephaly
    D. microphthalmia
    E. polydactyly

Answer 59

Jawab : B

Hypotelorism, not hypertelorism, is associated with trisomy 13.

Question 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

Answer 60

Jawab : A

Question 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

Answer 61

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.

Question 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

Answer 62

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.

Question 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

Answer 63

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.

Question 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

Answer 64

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.

Question 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

Answer 65

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.

Question 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

Answer 66

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.

Question 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

Answer 67

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.

Question 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

Answer 68

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.

Question 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

Answer 69

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.

Question 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

Answer 70

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.

Question 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.

Answer 71

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.

Question 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

Answer 72

Jawab : A

Cortical arteriovenous malformations (AVMs) are not characteristic of the Sturge-Weber syndrome.

Question 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.

Answer 73

Jawab : B

The demyelination, spongiosis, and gliosis seen in vitamin B12 deficiency are most common at lower cervical and thoracic levels.

Question 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

Answer 74

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.

Question 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

Answer 75

Jawab : E

Other associations are retinal hemangioblastomas, adrenal tumors, and polycythemia. The latter results from the production of a hematopoietic factor by the tumor.

Question 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

Answer 76

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.

Question 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

Answer 77

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.

Question 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

Answer 78

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.

Question 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.

Answer 79

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.

Question 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.

Answer 80

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.

Question 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

Answer 81

Jawab : B

Hemorrhage is also common in melanoma, renal cell carcinoma, and lung carcinoma.

Question 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

Answer 82

Jawab : C

Question 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.

Answer 83

Jawab : E

Question 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.

Answer 84

Jawab : D

Question 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

Answer 85

Jawab : B

Question 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.

Answer 86

Jawab : A

Question 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

Answer 87

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.

Question 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

Answer 88

Jawab : E

Vitamin A excess has not been associated with central pontine myelinolysis.

Question 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

Answer 89

Jawab : A

Rosenthal fibers, eosinophilic masses observed in astrocytic processes, are associated with pilocytic astrocytomas (neoplastic), and astrocytosis and Alexander’s disease (nonneoplastic).

Question 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

Answer 90

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.

Question 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

Answer 91

Jawab : A

Question 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

Answer 92

Jawab : A

The motor nuclei of cranial nerves V, VII, IX, and XI1 as well as the motor*cortex may be affected.

Question 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

Answer 93

Jawab : C

Cavernous hemangiomas are composed of large. thin-walled vessels without interposed brain parenchyma.

Question 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

Answer 94

Jawab : E

Question 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

Answer 95

Jawab : A

Question 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

Answer 96

Jawab : D

Question 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

Answer 97

Jawab : C

Question 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

Answer 98

Jawab : B

Question 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

Answer 99

Jawab : A

Question 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

Answer 100

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.

Question 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

Answer 101

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.

Question 102

60. High levels of alpha-fetoprotein are associated with
    A. endodermal sinus tumors
    B. choriocarcinomas
    C. germinomas
    D. pineoblastomas
    E. teratomas

Answer 102

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).

Question 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

Answer 103

Jawab : B

Question 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

Answer 104

Jawab : C

Question 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

Answer 105

Jawab : B

Question 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.

Answer 106

Jawab : D

Question 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

Answer 107

Jawab : E

Question 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

Answer 108

Jawab : A

Question 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

Answer 109

Jawab : C

Question 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

Answer 110

Jawab : A

Question 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

Answer 111

Jawab : B

Question 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

Answer 112

Jawab : C

Question 113

71. High levels of human chorionic gonadotrophin are seen in
    A. choriocarcinoma
    B. embryonal carcinoma
    C. endodermal sinus tumor
    D. germinoma
    E. teratoma

Answer 113

Jawab : A

Question 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

Answer 114

Jawab : B

Question 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

Answer 115

Jawab : C

Question 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

Answer 116

Jawab : D

Question 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

Answer 117

Jawab : B

Question 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

Answer 118

Jawab : E

Question 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

Answer 119

Jawab : A

Question 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

Answer 120

Jawab : A

Question 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.

Answer 121

Jawab : D

Question 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.

Answer 122

Jawab : C

Question 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.

Answer 123

Jawab : A

Question 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

Answer 124

Jawab : A

Question 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.

Answer 125

Jawab : E

Question 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

Answer 126

Jawab : B

Question 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

Answer 127

Jawab : C

Question 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.

Answer 128

Jawab : D

Question 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

Answer 129

Jawab : A

Question 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.

Answer 130

Jawab : E

Question 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

Answer 131

Jawab : D

Question 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

Answer 132

Jawab : A

Question 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

Answer 133

Jawab : B

Question 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

Answer 134

Jawab : E

Question 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

Answer 135

Jawab : A

Question 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

Answer 136

Jawab : D

Question 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

Answer 137

Jawab : A

Question 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

Answer 138

Jawab : C

Wilson’s disease is transmitted as an autosomal reces- sive trait.

Question 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

Answer 139

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.

Question 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

Answer 140

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.

Question 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

Answer 141

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.

Question 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

Answer 142

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.

Question 143

101. The most common neurologic complication of acquired immunodeficiency syndrome (AIDS) is
     A. dementia
     B. inflammatory polymyositis
     C. lymphoma
     D. myelopathy
     E. toxoplasmosis

Answer 143

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.

Question 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

Answer 144

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.

Question 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

Answer 145

Jawab : C

Hamartomas (not neurofibromas) of the iris (Lisch nod- ules) are szen in NF-I.

Question 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.

Answer 146

Jawab : C

Cerebral amyloid angiopathy is confined to intracranial arteries and arterioles in the leptomeninges and superficial cortex.

Question 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

Answer 147

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.

Question 148

106.

A. rarely multiple
B. associated with immunosuppression in older men
C. associated with immunosuppression in younger men
D. resistant to steroids

Answer 148

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.

Question 149

107.

A. Hirano bodies
B. inclusion bodies of subacute sclerosing panencephalitis (SSPE)
C. Lewy bodies
D. Pick’s bodies
E. rabies

Answer 149

Jawab : B

Question 150

108.

A. Hirano bodies
B. inclusion bodies of SSPE
C. Lewybodies
D. Pick’s bodies
E. rabies

Answer 150

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.

Question 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

Answer 151

Jawab : D

Pick’s bodies (rounded intracytoplasmic masses) are visible in the pyramidal neurons of the hippocampus.

Question 152

110.

A. inclusion bodies of HSV-l
B. Lewybodies
C. Pick’s bodies
D. rabies
E. inclusion bodies of SSPE

Answer 152

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.

Question 153

111.

A. Ganglioglioma
B. Hepatic encephalophaty
C. HSV-1
D. Parkinson’s disease
E. normal cortex

Answer 153

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.

Question 154

112.

A. Fibrillary astrocytoma
B. gemistrocytic astrocytoma
C. glioblastoma multiforme
D. hemangioblastoma
E. oligodendroglioma

Answer 154

Jawab : B

The cells of this variant of astrocytoma have prominent eosinophilic cytoplasm, short processes, and eccentric nuclei.

Question 155

113.

A. aneurysmal subarachnoid hemorrhage
B. bacterial meningitis
C. contusion
D. HSV-l
E. subdural hematoma

Answer 155

Jawab : D

The hemorrhagic appearance of the medial temporal lobe is characteristic of HSV-I.

Question 156

114.

A. AIDS encephalopathy
B. giant-cell glioblastoma multiforme (GBM)
C. hemangioblastoma
D. Creutzfeldt-Jakob disease
E. progressive multifocalleukoencephalopathy

Answer 156

Jawab : E

Demyelination and oligodendroglial cell loss are seen. Residual oligodendroglial nuclei are large and bizarre.

Question 157

115.

A. AIDS encephalopathy
B. giant-cell GBM
C. hemangioblastoma
D. Creutzfeldt-jakob disease
E. progressive multifocal leukoencephaltopathy

Answer 157

Jawab : D

Numerous vacuoles give a spongiform appearance.

Question 158

116.

A. metastatic disease
B. NF – 1
C. NF – 2
D. tuberous scelorosis
E. von hippel-Lindau disease

Answer 158

Jawab : B

The iris hamartomas (Lisch nodules) of NF-I are seen.

Question 159

117.

A. AIDS encephalopathy
B. giant-cell GBM
C. hemangioblastoma
D. Creutzfeldt-Jakob disease
E. progressive multifocalleukoencephalopathy

Answer 159

Jawab : A

Perivascular foci of inflammation with prominent multinu- cleated giant cells are typical.

Question 160

118.

A. anaplastic astrocytoma
B. ependymoma
C. ganglioglioma
D. meningioma
E. oligodendroglioma

Answer 160

Jawab : C

The finding of abnormal ganglion cells (including binucleate forms) is key to the diagnosis of ganglioglioma.

Question 161

119.

A. anaplastic astrocytoma
B. meningioma
C. normal pituitary gland
D. oligodendroglioma
E. pituitary adenoma

Answer 161

Jawab : D

CNBR Fig. 3-68. A “chicken wire” vascular pattern and a monotonous nuclear array are seen.