Multiple System Atrophy with Cerebellar Ataxia
Multiple system atrophy with predominant cerebellar ataxia (MSA-C) is a sporadic disorder which usually presents as a midline cerebellar disorder which progresses more rapidly than other late-onset sporadic ataxias. The patient typically becomes wheelchair-dependent within 5 years of onset. Diagnostic criteria for probable MSA-C include a sporadic, progressive adult-onset cerebellar syndrome together with additional features of parkinsonism, Babinski signs, and laryngeal stridor. Interestingly, cerebellar ataxia often improves as signs of parkinsonism emerge later in the disease. A recent consensus statement emphasizes neuroimaging features including atrophy of the putamen, middle cerebellar peduncle, or pons; FDG-PET hypometabolism in the putamen; and presynaptic nigrostriatal dopaminergic denervation with SPECT or PET imaging.
KeywordsMultiple System Atrophy Cerebellar Ataxia Spinocerebellar Ataxia Middle Cerebellar Peduncle Babinski Sign
Clip 1: the patient shows evidence of mild facial masking and parkinsonism, but the cerebellar features are more prominent including gait ataxia and inability to perform tandem gait. Clip 2: at follow-up examination 18 months later, facial masking has become more prominent, and the upper extremities and gait have become more ataxic. She now requires assistance to walk for a few steps and she has become wheelchair-dependent.
MSA-C.mp4 (MP4 31,298KB)