Objective: To assess whether MSA-specific MRI changes predict a more rapid clinical decline in a cohort of patients with a clinical diagnosis of MSA.

Background: It is currently unknown whether patients with clinically suspected MSA and MR features consistent with MSA differ in their progression from those patients who do not have MSA-specific MR changes.

Methods: 22 MSA patients who participated in previous prospective natural history studies and had a MR scan soon after their first clinic visit were followed for at least 48 months at our outpatient clinic. 12-month clinical progression was determined using the UMSARS. The following baseline MR abnormalities were assessed: putaminal, pontine, cerebellar and middle cerebellar peduncle atrophy, a putaminal hyperintense rim, putaminal signal hypointensity, and the hot cross bun sign. MR abnormalities were graded on a 4-point likert scale with increasing severity (no, mild, moderate, severe abnormality). Patients were stratified into two subgroups: (1) Patients with MR features consistent with MSA (MR-positive) and (2) patients without evidence of MSA-specific structural MR changes (MR-negative). MR-positivity was defined by the presence of at least one MR abnormality graded moderate or of more than two minor MR abnormalities. Clinical progression between the two groups were compared using an ANCOVA model which was corrected for baseline UMSARS score, age, gender and disease duration.

Results: 17 of 22 patients (77%) showed MR abnormalities consistent with MSA. There were no significant differences between the two groups in age, gender, disease duration at baseline visit or baseline UMSARS scores. Disease progression was significantly different between the two groups with MR-positive patients showing a more rapid clinical decline (UMSARS total mean difference MR-positive vs. MR-negative group: 21.7 [95% CI 16.4 – 27.1] vs. 9.3 [95% CI -1.0 – 19.6], F = 4.9, p = 0.041).

Conclusions: The presence of MR abnormalities has a significant impact on disease progression irrespective of clinical diagnosis, disease duration or baseline disease severity in MSA patients. This observation should be considered for the planning of future clinical trials.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/mri-supported-diagnosis-of-multiple-system-atrophy-implications-for-clinical-trials/