by Kurt Jellinger
A disease of the endothelium in small vessels giving rise to small subcortical infarcts and hemorrhages as well as to extensive white matter changes.
Hereditary multi-infarct dementia.
Symptoms and course
In contrast to the common forms of vascular dementia, patients are usually not hypertensive. Migraine attacks with aura (visual misperceptions) precede the onset of cognitive impairment.
Cognitive impairment and dementia symptoms are different from AD Stepwise decline in cognitive ability associated with minor strokes and persistent focal neurological symptoms.
Death occurs 15-25 years after the patients’ first stroke.
Causes and risk factors
Mutations on the NOTCH-3 gene cause inclusions in the smooth arterial muscles, the arterioles walls are thickened which impedes blood supply to the subcortial brain areas.
MRI demonstrates lacunar infarcts and leukoencephalopathy. A skin biopsy can demonstrate granular osmiophilic material.
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Last Updated: Friday 09 October 2009