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“Phalanx sign” helps to discriminate MSA-C from idiopathic late onset cerebellar ataxia

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Abstract

Introduction

Early diagnosis of MSA-C in patients with late-onset cerebellar ataxia (LOCA) may prove difficult. We therefore describe and evaluate the performance of the new “phalanx sign” (PS), that should be looked for during the nose–finger test to distinguish MSA-C from idiopathic late-onset ataxia (ILOCA).

Methods

Sensitivity, specificity, positive predictive value, negative predictive value and interrater reliability of PS were assessed in three groups: 21 MSA-C, 23 ILOCA and 20 age-matched healthy subjects.

Results

PS was positive for 61.9% of MSA-C patients’, 4.3% of ILOCA patients’ and in none of healthy subjects’ evaluations. PS discriminated MSA-C from ILOCA (p < 0.001) with a sensitivity of 61.9%, a specificity of 95.7%, a positive predictive value of 92.9%, a negative predictive value of 73.3% and a substantial interrater reliability (Kappa = 0.7273).

Conclusion

PS could be a helpful, easy and reproducible sign for the early diagnosis of MSA-C in patients with LOCA.

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Acknowledgements

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

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Authors and Affiliations

  1. Service de Neurologie, Centre Hospitalier Universitaire F. Mitterrand, Dijon, France

    Vincent Schneider

  2. Département de Neurologie, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1, Avenue Molière, 67098, Strasbourg Cedex, France

    Thomas Wirth, Andra Iosif, Solveig Montaut, Ouhaid Lagha-Boukbiza, Christine Tranchant & Mathieu Anheim

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

    Thomas Wirth, Andra Iosif, Solveig Montaut, Ouhaid Lagha-Boukbiza, Christine Tranchant & Mathieu Anheim

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

    Thomas Wirth, Andra Iosif, Solveig Montaut, Ouhaid Lagha-Boukbiza, Christine Tranchant & Mathieu Anheim

Authors
  1. Vincent Schneider
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  2. Thomas Wirth
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  3. Andra Iosif
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  4. Solveig Montaut
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  6. Christine Tranchant
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  7. Mathieu Anheim
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Corresponding author

Correspondence to Mathieu Anheim.

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Conflicts of interest

Mathieu Anheim declares honoraria and/or grants from AbbVie, Teva, Merz, Orkyn, Aguettant, Actelion Pharmaceuticals, Johnson and Johnson. Christine Tranchant declares honoraria from Merz and Allergan. Thomas Wirth received research grants from the Revue Neurologique, the Fondation Planiol and the APTES association and travel funding from LVL medical.

Ethical standards

Local ethics committee approved the study and all patients gave written consent for participation.

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Schneider, V., Wirth, T., Iosif, A. et al. “Phalanx sign” helps to discriminate MSA-C from idiopathic late onset cerebellar ataxia. J Neurol 269, 3900–3903 (2022). https://doi.org/10.1007/s00415-022-10994-3

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  • DOI: https://doi.org/10.1007/s00415-022-10994-3

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