Highlights
- •The imaging characteristics of the criteria-unfulfilled MSA-C at the initial stage
- •Decreased pontine area and significant laterality of MCP width of cerebral MRI
- •SPECT analysis revealed that the pontine rCBF was reduced.
- •Laterality of cerebellar rCBF and laterality of MCP width were positive correlated.
- •These could be useful to consider the possibility of criteria-unfulfilled MSA-C.
Abstract
To elucidate clinically useful imaging characteristics of multiple system atrophy
with predominant cerebellar ataxia (MSA-C) at the initial stage showing pure cerebellar
ataxia but unfilling consensus criteria (MSA-pc), clinical and neuroradiological analyses
on cerebral MRI and single-photon emission computed tomography (SPECT) for measuring
regional cerebral blood flow (rCBF) were performed. Seven MSA-pc patients meeting
the above condition at an initial evaluation were identified, and all the MSA-pc patients
later developed autonomic dysfunction and finally fulfilled the criteria for probable
or possible category of MSA-C. For comparison, two patients with spinocerebellar ataxia
type 6 and three patients with idiopathic cerebellar ataxia who did not exhibit autonomic
dysfunction for more than three years were enrolled in this study (non-MSA-pc). As
non-ataxic controls without cerebellar involvement, seven patients with Parkinson's
disease were also enrolled. As a result, MRI analysis clarified a smaller pontine
area and significant laterality of middle cerebellar peduncle (MCP) width in MSA-pc
in comparison to non-MSA-pc and controls. SPECT analysis revealed that pontine rCBF
was reduced even at the initial stage of MSA-pc. Moreover, the laterality of cerebellar
rCBF values and the laterality of MCP width in MSA-pc patients exhibited a significant
positive correlation, indicating anatomical and functional laterality of afferent
projections to cerebellum is a characteristic finding for MSA-pc. These neuroimaging
characteristics could be clinically useful to consider the possibility of the criteria-unfulfilled
MSA and promote an earlier intervention after obtaining a diagnosis of probable MSA-C.
Keywords
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Article info
Publication history
Published online: May 13, 2022
Accepted:
May 10,
2022
Received in revised form:
April 20,
2022
Received:
January 19,
2022
Identification
Copyright
© 2022 Elsevier B.V. All rights reserved.
