<% strPathPics = Session("strPathPicsL") imgBg = strPathPics + Session("strMedia") %> Spinocerebellar Degeneration

Spinocerebellar Ataxia - Case 1

A 33 year-old man presented with slowly progressive ataxia, slurred speech and nystagmus since childhood.

Show the Cerebellar Atrophy             Show the Pontine Atrophy            Show the Enlarged 4th Ventricle

Spinocerebellar Ataxia (SCA): T1-weighted axial MRIs. Note the severe atrophy of the cerebellum and pons. These disorders, once termed olivopontocerebellar atrophy (OPCA), comprise a large group of genetic disorders characterized by progressive ataxia and brainstem signs. There are now over 25 spinocerebellar ataxias. Inheritance includes autosomal-dominant and autosomal-recessive forms. Some spinocerebellar ataxias are due to tri-nucleotide repeats (akin to Huntington's Disease and Myotonic Dystrophy). Other neurological findings may include specific eye movement abnormalities, optic atrophy, macular degeneration, peripheral neuropathy, extrapyramidal findings, seizures, and cognitive impairment.

Revised 12/02/06
Copyrighted 2006. David C Preston