Objective: Diagnostic value of brain magnetic resonance imaging (MRI) in differentiating multiple system atrophy cerebellar (MSA-C) phenotype and spinocerebellar ataxias (SCAs) was assessed according to the disease duration.

Background: Clinical differentiation between MSA-C and SCAs is often challenging especially in the early stage. Although hot cross bun (HCB) signs and hyperintensities in the middle cerebellar peduncles (MCP signs) were commonly found on brain MRIs of MSA-C and SCAs, none of the published studies focused on the time when HCB and MCP signs appeared in the disease course.

Method: Among patients who had visited Samsung Medical Center in Seoul, 186 patients with probable MSA-C and 117 with genetically confirmed SCAs were included. T2-weighted brain MRIs were divided according to disease duration, and prevalence of HCB signs and MCP signs were analyzed.

Results: Baseline characteristics of the patients were comparable to those of previous studies. HCB and MCP signs were exclusively prevalent in MSA-C than in SCA at 3 years of disease duration. This distinction was even more evident with the coexistence of HCB and MCP signs. More patients with MSA-C were found to have HCB and MCP signs in the follow-up study. Among SCAs, HCB and MCP signs were most frequently observed in SCA2.

Conclusion: This study illustrates that MRIs in the early stage can differentiate MSA-C from SCAs, as HCB and MCP signs were prevalent and appeared earlier in MSA-C than in SCAs. Furthermore, more changes were observed in MSA-C than in SCAs as the disease progressed. The differential value of HCB and MCP signs in patients with ataxia should be interpreted in association with the disease duration.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/differential-value-of-brain-magnetic-resonance-imaging-in-msa-c-and-scas/