Clinical Neuropathology QUIZ #15

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Published in Clinical Neuropathology Vol 41 nr.5 (September-October 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/septemberoctober-12.html

Which of the following pathological alterations can be detected in semilobar holoprosencephaly?
(1 out of 5 is wrong – mark the wrong one)

1 point

a – Lack of optic nerves

b – Presence of leptomeningeal neuronal heterotopia

c – Herniation of cerebellar tonsils

d – Lack of olfactory structures

e – Fusion of basal ganglia and thalamus

2. Which of the following immunohistochemical markers may specifically indicate precursor cells of the pituitary gland?
(1 out of 5 is right – mark the correct one)

1 point

a – Cytokeratin

b – Olig-2

c – SSTR-2a

d – Synaptophysin

e – SF-1

3. The neuroprotective role of a chaperone in the nervous system is linked to clearance of the following intracellular proteins
(2 out of 5 are wrong – mark the wrong ones)

1 point

a – TDP-43

b – Ubiquitin

c – Huntingtin

d – SOD1

e – Alpha crystallin

4. Distal hereditary motor neuropathy comprises the following signs and symptoms
(2 out of 5 are wrong – mark the wrong ones)

1 point

a – Increased muscle tone

b – Symmetrical atrophy of calf muscles on MRI

c – Reduced compound muscle action potential of distal motor nerves

d – Paresthesia

e – Steppage gait

5. Amyotrophic lateral sclerosis (ALS): the following statements are true except for
(1 out of 5 is wrong – mark the wrong one)

1 point

a – Cytoplasmic accumulation of alpha synuclein in motor neurons is the hallmark of ALS

b – Approximately 10% of ALS cases are familial

c – There is considerable heterogeneity of age at onset and duration of disease

d – More than 200 pathogenic variants of SOD1 linked to familial ALS have been described

e – ALS is associated with loss of motor neurons in cerebral cortex, brainstem, and spinal cord

6. The following characteristics of disease are associated the spinocerebellar ataxia with axonal neuropathy (SCAN)
(1 out of 5 is wrong – mark the wrong one)

1 point

a – There is an autosomal recessive pattern of inheritance

b – Sensory nerve damage without motor nerve involvement is an early symptom

c – Nerve biopsy shows demyelinating disease

d – In the advanced phase of disease, MRI shows cerebellar atrophy

e – Nerve conduction studies indicate damage to axons

7. The following characteristics may indicate aggressive behavior in a pituitary adenoma
(1 out of 5 is wrong – mark the wrong one)

1 point

a – Ki-67 index > 10 %

b – Female sex of the patient

c – Negativity of Estrogen receptor expression

d – Positivity of p53 expression

e – Expression of SSTR-2a

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