Dysautonomia Is Frequent In Machado-joseph Disease: Clinical And Neurophysiological Evaluation.

dc.creatorTakazaki, Karen A G
dc.creatorD'Abreu, Anelyssa
dc.creatorNucci, Anamarli
dc.creatorLopes-Cendes, Iscia
dc.creatorFrança, Marcondes C
dc.date2013-Aug
dc.date2015-11-27T13:31:22Z
dc.date2015-11-27T13:31:22Z
dc.date.accessioned2018-03-29T01:17:14Z
dc.date.available2018-03-29T01:17:14Z
dc.identifierCerebellum (london, England). v. 12, n. 4, p. 513-9, 2013-Aug.
dc.identifier1473-4230
dc.identifier10.1007/s12311-013-0458-y
dc.identifierhttp://www.ncbi.nlm.nih.gov/pubmed/23413156
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/200493
dc.identifier23413156
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1300726
dc.descriptionAutonomic 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.
dc.description12
dc.description513-9
dc.languageeng
dc.relationCerebellum (london, England)
dc.relationCerebellum
dc.rightsfechado
dc.rights
dc.sourcePubMed
dc.subjectAdult
dc.subjectDiagnostic Techniques, Neurological
dc.subjectFemale
dc.subjectGalvanic Skin Response
dc.subjectHeart Rate
dc.subjectHumans
dc.subjectMachado-joseph Disease
dc.subjectMale
dc.subjectMiddle Aged
dc.subjectPrimary Dysautonomias
dc.titleDysautonomia Is Frequent In Machado-joseph Disease: Clinical And Neurophysiological Evaluation.
dc.typeArtículos de revistas


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