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

Cerebellar Degeneration

A 34 year woman old presented with slowly progressive ataxia since childhood.

Show the Cerebellar Atrophy       Show the Normal Pons      Show the Enlarged Cisterna Magna

(Left) T2 Axial MRI; (Middle) T1 Axial MRI; (Right) T1 Sagittal MRI. In all the scans, note the severe atrophy of the cerebellum.  Marked atrophy of the cerebellum is most often associated with genetic abnormalities, although severe cases of alcohol  intake as well as paraneoplastic conditions can also result in cerebellar atrophy. In this case, the patient was demonstrated to have adult onset hexosaminidase A deficiency (Late Onset Tay Sachs disease).

Revised 04/19/06.
The Electronic Curriculum is copyrighted 1998,  Case Western Reserve University School of Medicine.