Session Information
Date: Monday, September 23, 2019
Session Title: Ataxia
Session Time: 1:45pm-3:15pm
Location: Les Muses, Level 3
Objective: To evaluate, in patients with sporadic late-onset cerebellar ataxia (SLOCA), the discriminative value of each of the magnetic resonance imaging (MRI) features for the diagnosis of multiple system atrophy type cerebellar (MSA-C),to follow their evolution during the course of MSA-C, and to search correlations between each of these MRI features and clinical and paraclinical signs (DaTscan).
Background: The second consensus statement for the diagnosis of 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 sign have been described, and these MRI features have been rarely studied for the diagnostic of MSA-C in particular with population of cerebellar ataxia.
Method: Consecutive patients referred for cerebellar ataxia for SLOCA (age at onset > 40) underwent comprehensive clinical evaluation, 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 sign were more frequent in 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.
Conclusion: MCP hyperintensity and HCB sign should be added to MRI criteria for diagnosis of MSA-C. MRI signal abnormalities suggestive of MSA-C should be searched for in suitable sequence.
To cite this abstract in AMA style:
G. Carré, JL. Dietemann, O. Gebus, S. Montaut, O. Lagha-Boukbiza, T. Wirth, S. Kremer, IJ. Namer, M. Anheim, C. Tranchant. Brain MRI-based discrimination between multiple system atrophy type cerebellar from other late onset sporadic cerebellar ataxias: a prospective study with implications for diagnosis criteria [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/brain-mri-based-discrimination-between-multiple-system-atrophy-type-cerebellar-from-other-late-onset-sporadic-cerebellar-ataxias-a-prospective-study-with-implications-for-diagnosis-criteria/. Accessed June 14, 2025.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/brain-mri-based-discrimination-between-multiple-system-atrophy-type-cerebellar-from-other-late-onset-sporadic-cerebellar-ataxias-a-prospective-study-with-implications-for-diagnosis-criteria/