Pathology of CADASIL

Topic: Pathology

Created on Thursday, November 29 2007 by jdmiles

Last modified on Thursday, November 29 2007.

 
        A) Lymphocytic infiltration of the arteries
 
        B) Inflammation of the arteries, with multinucleated giant cells proliferating in the intimal layer
 
        C) Diffuse white matter disease with hemorrhages of various ages
 
        D) Thickened arteries with granular, periodic acid-Schiff-positive material in the media
 
        E) Neurtrophilic inflammation of the arteries
 

 

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This question was created on November 29, 2007 by jdmiles.
This question was last modified on November 29, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS

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A) Lymphocytic infiltration of the arteries

This answer is incorrect.

Arteries with mononuclear infiltration are characteristic of giant cell arteritis, but not of CADASIL.  (See References)

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B) Inflammation of the arteries, with multinucleated giant cells proliferating in the intimal layer

This answer is incorrect.

Arteries with multinucleated giant cells in the intima are characteristic of primary angiitis of the CNS, but not of CADASIL.  (See References)

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C) Diffuse white matter disease with hemorrhages of various ages

This answer is incorrect.

Hemorrhage is not common in CADASIL.  (See References)

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D) Thickened arteries with granular, periodic acid-Schiff-positive material in the media

This answer is correct.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic vascular disease associated with a defect on the Notch 3 gene on chromosome 19.

Typical pathological findings include lacunes in the white matter and deep cerebral nuclei, and thickening of the walls of the arteries of the parenchyma and leptomeninges. On light microscopy, the arterial walls contain granular material which stains with periodic acid-Schiff. On electron microscopy, this material is referred to as granular osmiophilic material (GOM).

  (See References)

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E) Neurtrophilic inflammation of the arteries

This answer is incorrect.

Arteries with neutrophilic inflammation are characteristic of polyarteritis nodosa, but not of CADASIL.  (See References)

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References:

1. Prayson, R.A., and Goldblum, J.R. (Eds.) (2005). Neuropathology. Elsevier Churchill Livingstone, Philadelphia. (ISBN:0443066582)

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pathology
Pathology of CADASIL
Question ID: 112907166
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 11/29/2007
Modified: 11/29/2007
Estimated Permutations: 1200