Question:

What is the pathophysiologic mechanism of CADASIL?
1. Chronic hypertension and arteriosclerosis.
2. Active inflammation.
3. Micro hemorrhage.
4. Ischemic infarcts.
5. Embolic infarcts.





Answer:

The correct answer for the question "What is the pathophysiologic mechanism of CADASIL?" is:

4. Ischemic infarcts.



Explanation
1. This is the mechanism for Binswanger disease. In CADASIL, there is osmiophillic deposition in the small vessel walls, resulting in chronic hypoperfusion, dysfunctional arterial autoregulation and more commonly lacunar infarcts without vessel stenosis. [These pathophysiologic phenomenon in CADASIL occur consequent to the destruction of vascular wall muscle cells and its subsequent failure to secrete vascular permeability factors, rather than luminal stenosis, as has identified in skin biopsies of CADASIL patients.]

2. Active inflammation of small vessel walls occurs as post infectious phenomenon and in vasculitides. [The missense mutation (more than 150 described) in CADASIL results in a nonfunctional NOTCH3 receptor that leads to accumulation of the pathognomonic granular osmiophilic material deposits within the cells and subsequent degeneration of smooth muscle fibers.]

3. Micro hemorrhages may occur in CADASIL and are not the underlying pathophysiologic mechanism. [MRI findings of increased microbleeds, lacunar infarcts and ventricular volume over time have been associated with progressive cognitive decline in executive function.]

4. Lacunar type infarcts predominate. [The missense mutation (more than 150 described) in CADASIL results in a nonfunctional NOTCH3 receptor that leads to accumulation of the pathognomonic granular osmiophilic material deposits within the cells and subsequent degeneration of smooth muscle fibers. This results in fibrosis and loss of vasomotor reactivity of small vessel walls leading to chronic hypoperfusion, dysfunctional arterial autoregulation and ischemic infarcts.]

5. Embolic infarcts in CADASIL have been reported in one case study without determination of a source and are not the pathophysiologic mechanism for CADASIL. 

[Lacunar type infarcts are considered by most authors as the predominant ischemic event in CADASIL.]



From the manuscript:
Acute Watershed Infarcts with Global Cerebral Hypoperfusion in Symptomatic CADASIL
Radiology Case. 2013 Mar; 7(3):8-15


This article belongs to the Neuro section.




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From the manuscript

Acute Watershed Infarcts with Global Cerebral Hypoperfusion in Symptomatic CADASIL

Free full text article: Acute Watershed Infarcts with Global Cerebral Hypoperfusion in Symptomatic CADASIL

Abstract
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common form of hereditary cerebral angiopathy. We present a case in which a pattern of diffusion signal change compatible with bihemispheric acute watershed infarcts occurred in a symptomatic patient demonstrating global hypoperfusion. To our knowledge, watershed infarcts in the clinical presentation of CADASIL have not been previously described.






References



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