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http://repositorio.unicamp.br/jspui/handle/REPOSIP/341701
Type: | Artigo |
Title: | Autonomic dysfunction in hereditary spastic paraplegia type 4 |
Author: | Gonzalez-Salazar, C. Takazaki, K. A. G. Martinez, A. R. M. Pimentel-Silva, L. R. Jacinto-Scudeiro, L. A. Nakagawa, E. Y. Murakami, C. E. Fujiwara Saute, J. A. M. Pedroso, J. L. Barsottini, O. G. P. Teive, H. A. G. Franca Jr., M. C. |
Abstract: | SPAST mutations are the most common cause of hereditary spastic paraplegia (SPG4-HSP), which is characterized by progressive lower limb weakness, spasticity and hyperreflexia. There are few studies about non-motor manifestations in this disease and none about autonomic involvement. Therefore, the aim was to determine the frequency and pattern of autonomic complaints in patients with SPG4-HSP, as well as to determine the clinical relevance and the possible factors associated with these manifestations. Thirty-four molecularly confirmed SPG4 patients were recruited in a multicenter cross-sectional study, of whom 26 underwent detailed neurophysiological testing (heart rate variability, sympathetic skin response and the Quantitative Sudomotor Axonal Reflex Test). The Scales for Outcomes in Parkinson's Disease - Autonomic Questionnaire (SCOPA-AUT) was applied to quantify the severity of autonomic symptoms. Were compared with 44 age- and gender-matched healthy controls using non-parametric tests. P values Results In the SPG4-HSP group, there were 18 men with a mean age of 47.7 +/- 12.6 years. SCOPA-AUT scores were similar between patients and controls (P = 0.238). Only the urinary domain subscore was significantly higher amongst patients (4 vs. 2.5, P = 0.05). Absent sympathetic skin response in the hands and feet was more frequent amongst patients (20% vs. 0%, P < 0.001, and 64% vs. 0%, P = 0.006, respectively). Quantitative Sudomotor Axonal Reflex Test responses were also smaller throughout all recording regions in the SPG4-HSP group. Our results indicate that SPG4-HSP patients have sudomotor dysfunction caused by damaged small post-ganglionic cholinergic fibers. Damage in SPG4-HSP extends to the peripheral nervous system |
Subject: | QSART |
Country: | Reino Unido |
Editor: | Wiley |
Rights: | Fechado |
Identifier DOI: | 10.1111/ene.13878 |
Address: | https://onlinelibrary.wiley.com/doi/full/10.1111/ene.13878 |
Date Issue: | 2019 |
Appears in Collections: | FCM - Artigos e Outros Documentos |
Files in This Item:
File | Description | Size | Format | |
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000460307300022.pdf | 401.17 kB | Adobe PDF | View/Open |
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