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CASE NUMBER 319
Diagnosis

Multisystem Atrophy

Note
38-year-old male with a history of supranuclear palsy, postural instability, and mild dementia. These images demonstrate a hot cross bun sign consisting of bands of T2 hyperintense signal in the coronal and sagittal planes on the axial T2/fluid sensitive images. There is reduced volume and flattening of the anterior pons. Imaging findings are classic for multiple system atrophy. Multiple system atrophy is a sporadic neurodegenerative disorder demonstrating variable cerebellar ataxia, autonomic dysfunction, parkinsonism and corticospinal dysfunction. Clinical presentations include one of 3 patterns such as Shy Drager syndrome or MSA-A, striatonigral degeneration or MSA-P, and olivopontocerebellar atrophy or MSA-C. No effective treatment exists at this time. Death usually occurs within 10 years of diagnosis.
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