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Type: Artigo de periódico
Title: Dysautonomia Is Frequent in Machado-Joseph Disease: Clinical and Neurophysiological Evaluation
Author: Takazaki, KAG
D'Abreu, A
Nucci, A
Lopes-Cendes, I
Franca, MC
Abstract: Autonomic dysfunction has been already described in patients with SCA3/MJD, but several important questions remain unanswered. The objectives of this study are to determine the frequency and the intensity of autonomic manifestations in SCA3/MJD, as well as to identify possible correlations between autonomic manifestations and genetic and clinical parameters. We have performed clinical and electrophysiological evaluations of 40 patients with SCA3/MJD and 38 healthy controls. We used the Scale for the Assessment and Rating of Ataxia (SARA) and the scales for Outcomes in Parkinson's Disease: Autonomic Questionnaire to quantify the severity of ataxia and autonomic complaints, respectively. We also studied heart rate variability at rest, during orthostatic challenge (30:15 ratio), Valsalva maneuver (Valsalva index), and deep breathing (E/I ratio). We evaluated spectral analyses of RR intervals at rest and the sympathetic skin response. Mean RR intervals at rest and the 30:15 ratio were different between patients and controls (811.8 versus 933.4 ms; p = 0.001 and 1.10 versus 1.15; p = 0.038, respectively). The Valsalva index and the E/I ratio were similar between the groups (p = 0.373 and p = 0.08). Spectral analysis presented distinct results in patients and controls, related to low- and high-frequency power (p < 0.001 and < 0.001, respectively). We found cardiovascular and sympathetic sweat disautonomia in 30 % and 45 % of the patients with SCA3/MJD. Autonomic manifestations were related neither to genetic (CAG repeat length) nor clinical parameters (age, disease duration, SARA scores). Autonomic dysfunction is frequent and sometimes disabling in SCA3/MJD. We found evidence of both cardiovascular and sudomotor dysfunction in the disease.
Subject: SCA3
Machado-Joseph disease
Autonomic nervous system
Heart rate variability
Country: EUA
Editor: Springer
Citation: Cerebellum. Springer, v. 12, n. 4, n. 513, n. 519, 2013.
Rights: fechado
Identifier DOI: 10.1007/s12311-013-0458-y
Date Issue: 2013
Appears in Collections:Unicamp - Artigos e Outros Documentos

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