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

Vincent Schneider , Thomas Wirth (1, 2) , Andra Iosif , Solveig Montaut , Ouhaid Lagha-Boukbiza , Christine Tranchant (1, 2) , Mathieu Anheim (2, 1)

1 IGBMC - Institut de Génétique et de Biologie Moléculaire et Cellulaire
2 IGBMC - Institut de génétique et biologie moléculaire et cellulaire

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.

Domaines: Biochimie, Biologie Moléculaire

Vincent Schneider, Thomas Wirth, Andra Iosif, Solveig Montaut, Ouhaid Lagha-Boukbiza, et al.. "Phalanx sign" helps to discriminate MSA-C from idiopathic late onset cerebellar ataxia. Journal of Neurology, 2022, 269 (7), pp.3900-3903. ⟨10.1007/s00415-022-10994-3⟩. ⟨hal-04225543⟩