Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) Syndrome: A Case Report
Journal Title: Online Journal of Neurology and Brain Disorders - Year 2018, Vol 1, Issue 1
Abstract
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 aforementioned 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.
Authors and Affiliations
Suprakash Chaudhury, Rakesh Kumar Singh, Samiksh Sahul, Adnan Kadiyani
Keywords
Related Articles
- EP ID EP589359
- DOI 10.32474/OJNBD.2018.01.000101
- Views 94
- Downloads 0
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