Clinical Neuropathology QUIZ #9

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Published in Clinical Neuropathology Vol 40 nr.5 (September/October 2021). Based on papers and editorial of Clinical Neuropathology of the same issue.

1. Which of the following is a feature of Menke's disease? *

1 分

a - cerebral atrophy

b - cerebellar atrophy

c - tortuous meningeal vessels

d - torpedo-shaped axonal filaments

e - all of the above

2. Which of the following genes is implicated in Menke's disease? *

1 分

a - ATP7A

b - ATP7B

c - SERPINA1

d - HEXA

e - PRKN

3. What is the most common location of atypical teratoid rhabdoid tumor occurring in adults? *

1 分

a - cerebellum

b - midbrain

c - lateral ventricle

d - sellar region

e - temporal lobe

4. Which of the following genes is implicated in atypical teratoid rhabdoid tumor? *

1 分

a - NF1

b - TSC1

c - SMARCB1

d - FGFR1

e - KLF4

5. The histomorphology of polymorphous low-grade neuroepithelial tumor of the young resembles which tumor? *

1 分

a - oligodendroglioma

b - astrocytoma

c - medulloblastoma

d - meningioma

e - ependymoma

6. Which of these genes is often mutated in polymorphous low-grade neuroepithelial tumor of the young? *

1 分

a - ATRX

b - IDH1

c - IDH2

d - BRAF

e - SMARCB1

7. Which of the following findings may help to distinguish metastatic neuroendocrine carcinoma from PNET? *

1 分

a - lack of GFAP staining

b - expression of neuroendocrine markers

c - expression of cytokeratins

d - Reticulin staining

e - none of the above

8. Which of the following statements regarding mitochondrial myopathies is NOT true? *

1 分

a - presents with hypotonia

b - the clinical diagnosis can be histologically confirmed in the vast majority of cases

c - is associated with lactic acidosis

d - muscle biopsies feature dark fibers on SDH staining

e - muscle biopsies feature ragged red fibers

9. Which of the following is NOT a feature of chronic progressive external ophthalmoplegia? *

1 分

a - ptosis

b - proxinal muscle weakness

c - external ophthalmoplegia

d - trinucleotide expansion in the PABN1 gene

e - COX -negative muscle fibers

10. Which of the following pituitary adenomas is associated with aggressive clinical behavior? *

1 分

a - gonadotropic adenoma

b - thyreotropic adenoma

c - Crooke cell adenoma

d - densely granulated somatotropic adenoma

e - lactotropic adenoma occurring in young women

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