Multiple system atrophy parkinsonian type (MSA-P), previously known as striatonigral degeneration, is a neurodegenerative disease, and one of the phenotypes of multiple systemic atrophy (MSA).
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Terminology
The prevailing thought is that that olivopontocerebellar degeneration, Shy-Drager syndrome and striatonigral degeneration are different manifestations of the same underlying disease, namely multiple systemic atrophy (MSA). However, many older publications will describe these as separate entities 1,2.
For a discussion of epidemiology, pathology and diagnosis, see: multiple systemic atrophy (MSA).
Clinical presentation
MSA-P presents predominantly with parkinsonism with mild cerebellar and pyramidal and signs. Autonomic dysfunction is also common.
Unlike the other clinical phenotype (MSA-C), ataxia and bulbar symptoms are less evident.
Radiographic features
MRI is the imaging of choice in patients suspected of MSA. A diagnosis is made with a combination of clinical and MRI findings, although a negative MRI does not exclude MSA 5. Significant overlap of imaging findings exists between MSA-C and MSA-P 5.
MRI
Putaminal findings in MSA-P 3:
reduced volume
reduced signal relative to globus pallidus on susceptibility weighted sequences (T2*, GRE or SWI)
reduced signal relative to the red nucleus on susceptibility weighted sequences (T2*, GRE or SWI)
putaminal rim sign: abnormal high T2 linear rim surrounding the putamen at 1.5 T (this is a normal finding at 3T) 3-5
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