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
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 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