Quiz in Clinical Neuropathology

As of June 2020, each issue of Clinical Neuropathology will have a quiz, with questions based on the papers and the editorial of the previous issue.

QUIZ #1, JUNE 2020

The questions are repeated here. The solutions are written at the end.

Clinicopathological concordance in cognitive disease diagnostics

1. Which of the following conditions has the lowest clinicopathological concordance in the Study conducted by Andersson et. Al.?
a – Lewy Body Dementia
b – Alzheimers Disease
c – Mixed Dementia
d – Vascular Dementia
e – Creutzfeldt-Jakob Disease

2. All of the following statements regarding clinicopathological concordance as reported by Andersson et al in cognitive disease are valid EXCEPT
a – Full Clinicopathological Concordance was found in close to two thirds of the cases
b – The clinicopathologic concordance for a diagnosis of “mild cognitive impairment” could not be determined for lack of a defined neuropathological correlate
c – The high concordance for FTLD may be due to selective referral and high level of expertise at the center which conducted the study
d – In Alzheimer’s Disease, the clinicopathological concordance reached more than 50%
e – The clinicopathological concordance in cognitive disease diagnostics has improved over the last decades

MicroRNA and HDAC4 Protein expression in the skeletal muscle of ALS patients

3. Which of the following statements regarding ALS is true?
a – Neurodegeneration in ALS may be caused by a dysfunction in RNA metabolism pathomechanism may be an impairment in RNA-regulation.
b – Most cases of ALS are sporadic.
c – RNA-binding proteins such as TDP43, FUS and hnRNP a1 are implicated in ALS pathology.
d – More than 100 mutations of the SOD1 gene have been linked to ALS pathology
e – All of the above

4. All of the following observations on miRNA and HDAC4 Protein expression in the skeletal muscle of ALS patients have been made by Pegoraro et al EXCEPT

a -In sporadic ALS there is a decrease in expression HDAC4 protein
b – The expression of least one of the inflammatory/apoptotic miRNAs was observes in all ALS cases when compared to control cases
c – There is an upregulation of miR-206 in SOD1-ALS and C9-ALS
d – Muscle specific-miRNAs and inflammatory miRNAs are differentially expressed in different forms of ALS
e – There was no difference in the expression of miR133a and miR-133b in ALS compared to the control group

Cystic sellar salivary gland-like lesions

5. Which of the following statements about heterotopic salivary gland tissue is correct?
a – Salivary gland tissues in the pituitary gland are referred to as “Walthard cell nests”
b – Heterotopic salivary gland tissue exclusively occurs in the pituitary gland
c – Heterotopic salivary gland tissues in the pituitary gland are clinically symptomatic in most cases
d – Heterotopic salivary gland tissues are neoplastic in nature
e – Tumours such as oncocatoma and pleomorphic adenoma may arise from heterotopic salivary gland tissue

6. All of the following statements regarding Cystic sellar salivary gland-like lesions are correct EXCEPT
a- CSSL features emorphous eosinophilic colloid, cuboidal epithelial lining and salivary gland acini
b – The salivary gland tissue in CSSL consists exclusively of serous glands
c – CSSL may have display oncocytic change of the salivary gland acini
d – CSSL may present with endocrinologic abnormalities
e – none of the above

High grade glioma with SOS1-amplification

7. Which of the following assertions regarding MAPK signalling in gliomas is correct?
a – The MAPK signalling pathway is exclusively activated by increased phosphorylation at receptor tyrosine kinases
b -EGFR amplification leads to increased MAPK signalling
c – In pilocytic astrocytomas, the KIAA1549-BRAF fusion leads to inactivation of BRAF
d – Altered MAPK signalling in pilocytic astrocytomas is due to a BRAF mutation in most cases
e – Activating mutations of EGFR have not been described in gliomas

8. In the case of a high grade glioma reported by Vidal et al, all of the following observations were made EXCEPT
a – The authors report a high-grade glioma with necrosis and vascular proliferation adjacent to a low grade component with glioneuronal morphology
b – Amplification of SOS1 at 2p22.1 was seen
c – The amplification of the SOS1 gene was found in both the high grade and the low grade component
d – Microarray based comparative genomic hybridization revealed a loss of chromosome 1 with deletion of PTEN
e – aCGH revealed a copy gain at 5p15.33 containg TERT

High mitotic activity in a capillary hemangioma of the cauda equina

9. Which of the following statements regarding vascular lesions of the spinal cord is correct?
a – Less than 10% of intradural lesions are of vascular origin
b – Hemorrhage of a vascular lesion may lead to acute onset of neurologic symptoms
c – Spinal vascular tumors include arteriovenous malformations, teleangiectasis, capillary hemangiomas, and cavernous angiomas.
d – all of the above
e – none of the above

10. All of the following statements regarding intradural capillary hemangiomas are correct EXCEPT
a – Capillary hemangiomas are typically lobular in architecture
b – The vascular spaces are lined by CD31-positive endothelial cells
c – Intradural capillary hemangiomas, much like their counterpart of skin and soft tissue, are often associated with high mitotic activity
d – On MRI imaging, capillary hemangioma may appear similar to schwannoma and meningioma
e – Intradural capillary hemangiomas have no obvious gender predilection

Answers:

1d
2e
3e
4a
5e
6b
7b
8d
9d
10c