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- Title
Dysautonomia Is Frequent in Machado-Joseph Disease: Clinical and Neurophysiological Evaluation.
- Authors
Takazaki, Karen; D'Abreu, Anelyssa; Nucci, Anamarli; Lopes-Cendes, Iscia; França, Marcondes
- 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.
- Subjects
DYSAUTONOMIA; NEUROPHYSIOLOGY; ELECTROPHYSIOLOGY; PARKINSON'S disease; HEALTH outcome assessment; HEART beat; NERVOUS system
- Publication
Cerebellum, 2013, Vol 12, Issue 4, p513
- ISSN
1473-4222
- Publication type
Article
- DOI
10.1007/s12311-013-0458-y