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Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria

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Abstract

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.

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Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author information

Authors and Affiliations

  1. Service de Neurologie, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1 avenue Molière, 67098 Cedex, Strasbourg, France

    G. Carré, O. Gebus, S. Montaut, O. Lagha-Boukbiza, T. Wirth, M. Anheim & C. Tranchant

  2. Service de Neurologie, Hôpitaux Civils de Colmar, Hôpital Louis Pasteur, 39 avenue de la Liberté, 68024, Colmar, France

    G. Carré

  3. Service d’imagerie 2, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1 avenue Molière, 67098 Cedex, Strasbourg Cedex, France

    J. L. Dietemann & S. Kremer

  4. Service de Médecine Nucléaire, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1 avenue Molière, 67098 Cedex, Strasbourg, France

    I. J. Namer

  5. Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France

    M. Anheim & C. Tranchant

  6. Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), INSERM-U964/CNRS-UMR7104/Université de Strasbourg, Illkirch, France

    M. Anheim & C. Tranchant

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  1. G. Carré
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  2. J. L. Dietemann
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  3. O. Gebus
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  4. S. Montaut
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  5. O. Lagha-Boukbiza
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  6. T. Wirth
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  7. S. Kremer
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  8. I. J. Namer
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  9. M. Anheim
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  10. C. Tranchant
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Corresponding author

Correspondence to C. Tranchant.

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Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical approval

This study has been approved by the local ethical committee.

Electronic supplementary material

Below is the link to the electronic supplementary material.

415_2020_9702_MOESM1_ESM.pdf

Figure 1: Scale for the Assessment and Rating of Ataxia (SARA) with disease duration in group 1. Each point is the average value obtained when considering all the data obtained for MSA-C patients (PDF 10 kb)

415_2020_9702_MOESM2_ESM.pdf

Figure 2: Spinocerebellar Degeneration Functional Score (SDFS) with disease duration in group 1. Each point is the average value obtained when considering all the data obtained for MSA-C patients. (PDF 9 kb)

415_2020_9702_MOESM3_ESM.pdf

Figure 3: Unified Parkinson’s Disease Rating Scale III (UPDRS III) with disease duration in group 1. Each point is the average value obtained when considering all the data obtained for MSA-C patients. (PDF 10 kb)

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Carré, G., Dietemann, J.L., Gebus, O. et al. Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria. J Neurol 267, 1269–1277 (2020). https://doi.org/10.1007/s00415-020-09702-w

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  • DOI: https://doi.org/10.1007/s00415-020-09702-w

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