Clinical Neuropathology QUIZ #16

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Published in Clinical Neuropathology Vol 41 nr.6 (November-December 2022). Based on papers and editorial of Clinical Neuropathology of the same issue: https://www.dustri.com/nc/journals-in-english/mag/clinical-neuropathology/vol/volume-41-2022/issue/novemberdecember-12.html

1. Which of the following inheritance patterns has NOT been described in Charcot-Marie-Tooth disease?

1 point

a - autosomal dominant

b - autosomal recessive

c - X-linked dominant

d - mitochondrial

e - none of the above

2. Which of the following is NOT a typical feature of argyrophilic grain disease?

1 point

a - coiled bodies

b - ballooned neurons

c - Lewy bodies

d - argyrophilic grains

e - deposition of hyperphosphorylated tau-protein

3. Which of the following regions of the brain is typically affected first in argyrophilic grain disease?

1 point

a - medial temporal lobe

b - frontal lobe

c - basal ganglia

d - tectum mesencephali

e - cerebellum

4. Which of the following is a typical feature of IgG-related disease?

1 point

a - storiform fibrosis

b - lymphoplasmacytic infiltrate

c - obliterative phlebitis

d - elevated numbers of IgG4-positive plasma cells

e - all of the above

5. Which of the following immunohistochemical stains is NOT expected to be positive in lymphoplasmacytic infiltrates in IgG4-related disease?

1 point

a - BRAF(V600E)

b - CD79a

c - CD138

d - IgG

e - IgG4

6. Which of the following immunohistochemical stains is typically negative in diffuse midline glioma, H3K27-altered?

1 point

a - H3K27M

b - IDH1(R132H)

c - GFAP

d - Olig2

e - S100

7. Which is the most common localization of diffuse midline glioma, H3K27-altered?

1 point

a - vertebral bone

b - pons

c - spinal cord

d - occipital lobe

e - frontal lobe

8. Which of the following immunohistochemical stains is typically positive in histiocytic sarcoma?

1 point

a - synaptophysin

b - CD21

c - CD163

d - HMB45

e - SSTR2A

9. Which of the following diseases may feature onion bulb formation in nerve biopsies?

1 point

a - Charcot-Marie-Tooth disease

b - Dejerine-Sottas disease

c - chronic inflammatory demyelinating polyneuropathy

d - diabetic neuropathy

e - all of the above

10. Aminoacyl-tRNA synthetase binds which of the following nucleic acids?

1 point

a - tRNA

b - mRNA

c - siRNA

d - rRNA

e - all of the above

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