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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Sudomotor dysfunction is frequent and correlates with disability in Friedreich ataxia

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Author(s):
Takazaki, Karen A. G. [1] ; Rezende, Thiago Junqueira R. [1] ; Martinez, Alberto R. M. [1] ; Gonzalez-Salazar, Carelis [1] ; Nucci, Anamarli [1] ; Lopes-Cendes, Iscia [2] ; Franca, Jr., Marcondes C. [1]
Total Authors: 7
Affiliation:
[1] Univ Campinas UNICAMP, Sch Med Sci, Dept Neurol, Campinas, SP - Brazil
[2] Univ Campinas UNICAMP, Sch Med Sci, Med Genet, Campinas, SP - Brazil
Total Affiliations: 2
Document type: Journal article
Source: CLINICAL NEUROPHYSIOLOGY; v. 129, n. 11, p. 2290-2295, NOV 2018.
Web of Science Citations: 1
Abstract

Objectives: To evaluate autonomic symptoms and function in Friedreich's Ataxia (FRDA). Methods: Twenty-eight FRDA patients and 24 controls underwent clinical/electrophysiological testing. We employed the Friedreich's Ataxia Rating Scale (FARS) and the Scales for Outcomes in Parkinson's Disease: Autonomic Questionnaire-SCOPA-AUT to estimate the intensity of ataxia and autonomic complaints, respectively. Cardiovagal tests and the quantitative sudomotor axonal reflex, QSART, were then assessed in both groups. Results: In the patient group, there were 11 men with mean age of 31.5 +/- 11.1 years. Mean SCOPA-AUT score was 15.1 +/- 8.1. Minimum RR interval at rest was shorter in the FRDA group (Median 831.3 x 724.0 ms, p < 0.001). The 30:15 ratio, Valsalva index, E:I ratio, low and high frequency power presented no differences between patients and controls (p > 0.05). Sweat responses were significantly reduced in patients for all sites tested (forearm 0.389 x 1.309 mu L; proximal leg 0.406 x 1.107 mu L; distal leg 0.491 x 1.232 mu L; foot 0.265 x 0.708 mu L; p value < 0.05). Sweat volumes correlated with FARS scores. Conclusions: We found abnormal sudomotor but normal heart rate variability in FRDA. Small cholinergic post-ganglionic fibers are affected in the disease. Significance: Quantification of sudomotor function might be a biomarker for FRDA. (C) 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved. (AU)

FAPESP's process: 13/01766-7 - Sensory neuronopathies: investigation of new diagnostic methods, mechanisms of the disease and therapeutic strategies
Grantee:Marcondes Cavalcante Franca Junior
Support type: Research Grants - Young Investigators Grants