Cerebral Autosomal Dominant Arteriopathy withSubcortical Infarcts and Leukoencephalopathy(CADASIL) Syndrome: A Case Report by Rakesh Kumar Singh in Online Journal of Neurology and Brain Disorders(OJNBD)- Lupine Publishers
Cerebral autosomal dominant arteriopathy with sub cortical infarcts
and leukoencephalopathy (CADASIL) is a rare autosomal dominant genetic
disease. A35 year old women presented with progressive loss of memory
left sided weakness and hemiplegic and non insulin dependent diabetes
mellitus with characteristic MRI findings is reported due to its rarity.Cerebral small vessel disease (SVD) is increasingly being recognized
as the cause of stroke, impaired cognitive function and mood disorders
in geriatric age group. Commonly SVD is sporadic due to old age and high
blood pressure, but occasionally SVD has a monogenic cause. The best
known among these is cerebral autosomal dominant arteriopathy with sub
cortical infarcts and leuko encephalopathy(CADASIL), a rare type of
autosomal dominant cerebral angiopathy. It causes recurrent sub cortical
ischemic events and vascular dementia which appear as diffuse white
matter abnormality on neuro imaging. It involves mainly the small
cerebral vessels; histopathology reveals non-atherosclerotic and
non-amyloidal angiopathy. [1] The 1st case of a forementioned symptoms
was reported 30 years ago in a Swedish family [2] and the term CADASIL
was coined in early 1990's [3] Since then CADASIL has been diagnosed in
number of families and ethnic groups all over the world.
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