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Ataxia-telangiectasia also known as Louis-Bar syndrome, is a hereditary autosomal recessive progressive multisystem disease. The vermian atrophy is more prominent than the atrophy of the cerebellar hemispheres and is characteristic of the type of anatomic appearance seen in ataxia-telangiectasia. Also with age the degree of atrophy progresses, there are two siblings with more marked atrophy in later age group.
Ataxia-telangiectasia-MRI Reviewed by Sumer Sethi on Tuesday, June 29, 2010 Rating: 5

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