Olivopontocerebellar degeneration (MSA-C)

Imaging
biomarkers in Parkinson’s disease and Parkinsonian syndromes: current and emerging concepts. MRI of a patient with a clinical diagnosis of Multiple System Atrophy-C. a Axial proton density sequence at the level of the pons demonstrates cruciform pontine T2 hyperintensity consistent with the ‘hot cross bun’ sign secondary to selective vulnerability of the pontocerebellar tract in Multiple System Atrophy-C. Disproportionate atrophy of the pons and partially visualized cerebellar hemispheres is also evident. b Axial FLAIR sequence with cruciform T2 hyperintensity within the pons and middle cerebellar peduncles (i.e., ‘Middle Cerebellar Peduncle sign’) with marked atrophy. Cerebellar hemispheric and vermian atrophy is also seen with ex vacuo dilatation of the fourth ventricle. c Sagittal T1 sequence showing marked atrophy of the brainstem and cerebellar vermis
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Olivopontocerebellar degeneration (OPCD) 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 .

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).

Siehe auch:
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Assoziationen und Differentialdiagnosen zu Olivopontozerebelläre Atrophie: