Vincent Schneider1, Thomas Wirth2,3,4, Andra Iosif2,3,4, Solveig Montaut2,3,4, Ouhaid Lagha-Boukbiza2,3,4, Christine Tranchant2,3,4, Mathieu Anheim5,6,7. 1. Service de Neurologie, Centre Hospitalier Universitaire F. Mitterrand, Dijon, France. 2. Département de Neurologie, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1, Avenue Molière, 67098, Strasbourg Cedex, France. 3. Institut de Génétique Et de Biologie Moléculaire Et Cellulaire (IGBMC), UMR7104, INSERM-U964/CNRS, Université de Strasbourg, Illkirch, France. 4. Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France. 5. Département de Neurologie, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1, Avenue Molière, 67098, Strasbourg Cedex, France. mathieu.anheim@chru-strasbourg.fr. 6. Institut de Génétique Et de Biologie Moléculaire Et Cellulaire (IGBMC), UMR7104, INSERM-U964/CNRS, Université de Strasbourg, Illkirch, France. mathieu.anheim@chru-strasbourg.fr. 7. Fédération de Médecine Translationnelle de Strasbourg (FMTS), Université de Strasbourg, Strasbourg, France. mathieu.anheim@chru-strasbourg.fr.
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.
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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