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Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria
G Carré1,2, J L Dietemann3, O Gebus1
1Service de Neurologie, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1 avenue Molière, 67098 Cedex, Strasbourg, France.
Aim:
The second consensus statement for the diagnosis of multiple system atrophy type cerebellar (MSA-C) includes pons and middle cerebellar peduncle (MCP) atrophy as MRI features. However, other MRI abnormalities such as MCP hyperintensity, hot cross bun sign (HCB), putaminal hypointensity and hyperintense putaminal rim have been described.
Objectives:
To evaluate, in patients with sporadic late-onset cerebellar ataxia (SLOCA), the discriminative value of several MRI features for the diagnosis of MSA-C, to follow their evolution during the course of MSA-C, and to search for correlations between these MRI features and clinical signs.
Methods:
Consecutive patients referred for SLOCA underwent comprehensive clinical evaluation and laboratory investigations, brain MRI, DaTscan and a 1-year follow-up.
Results:
Among 80 patients, 26 had MSA-C, 22 another diagnosis, and 32 no diagnosis at the end of the follow-up. At baseline, MCP hyperintensity and HCB were more frequent in patients finally diagnosed with MSA-C than in other patients with SLOCA (p < 0.0001), and had the highest specificity (98.5%) and positive predictive value (91.7%) for the diagnosis of MSA-C, compared to all other MRI signs. The most relevant MRI sequence regarding HCB sign was the T2-proton density (DP) weighted. All MRI features were more frequent with disease duration. No correlation was found between any MRI feature and neither clinical data, nor dopaminergic neuronal loss (p = 0.5008), except between vermis atrophy and UPDRSIII score.
Conclusion:
MCP hyperintensity and HCB sign should be added into the list of additional features of possible MSA-C. MRI signal abnormalities suggestive of MSA-C should be searched for in suitable sequence.
Insights
Middle cerebellar peduncle (MCP) hyperintensity and the hot cross bun sign (HCB) are valuable MRI indicators for diagnosing multiple system atrophy type cerebellar (MSA-C) in patients with sporadic late-onset cerebellar ataxia (SLOCA). These findings aid in early and accurate MSA-C diagnosis.
Area of Science:
- Neurology
- Radiology
- Neuroimaging
Background:
- Consensus statements for multiple system atrophy type cerebellar (MSA-C) diagnosis include pons and middle cerebellar peduncle (MCP) atrophy.
- Other MRI abnormalities like MCP hyperintensity and the hot cross bun sign (HCB) have been described in MSA-C.
Purpose of the Study:
- To assess the diagnostic value of MRI features for MSA-C in patients with sporadic late-onset cerebellar ataxia (SLOCA).
- To track the evolution of these MRI features during MSA-C progression.
- To explore correlations between MRI findings and clinical symptoms.
Main Methods:
- Consecutive SLOCA patients underwent clinical evaluation, laboratory tests, brain MRI, and DaTscan.
- A 1-year follow-up was conducted for all participants.
Main Results:
- MCP hyperintensity and HCB sign were significantly more frequent in diagnosed MSA-C patients (p < 0.0001).
- These signs demonstrated high specificity (98.5%) and positive predictive value (91.7%) for MSA-C diagnosis.
- MRI abnormalities increased with disease duration; vermis atrophy correlated with UPDRSIII score.
Conclusions:
- MCP hyperintensity and HCB sign are valuable additional features for diagnosing possible MSA-C.
- Specific MRI sequences should be utilized to detect these suggestive signal abnormalities.
