Multiple system atrophy cerebellar type (MSA-C) also known as olivopontocerebellar degeneration is a neurodegenerative disease, and one of the clinical manifestations of multiple systemic atrophy (MSA).
It is important to note that the current belief that olivopontocerebellar degeneration, Shy-Drager syndrome, and striatonigral degeneration are different manifestations of the same underlying disease, namely multiple systemic atrophy (MSA), is recent, and as such many older publications will describe these as separate entities 2,3.
For a discussion of epidemiology and pathology, please refer to: multiple systemic atrophy (MSA).
Clinical presentation
Olivopontocerebellar degeneration presents predominantly with cerebellar and brainstem symptoms and signs. Typically ataxia and bulbar dysfunction are pronounced. Given the predominance of cerebellar symptoms, it is classified under the MSA-C subtype of MSA.
Unlike Shy-Drager syndrome and striatonigral degeneration (the other two manifestations of MSA), autonomic dysfunction and parkinsonism are less prominent.
Radiographic features
MRI
There is disproportionate atrophy of the cerebellum and brainstem (especially olivary nuclei and middle cerebellar peduncle) and T2 hyperintensities in certain locations 4.
T2: hyperintensities of the pontocerebellar tracts, middle cerebellar peduncles (middle cerebellar peduncle sign), pons (hot cross bun sign) 4
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