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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2016; 74(10): 796-802
DOI: 10.1590/0004-282X20160122
ARTICLE

Autonomic thermoregulatory dysfunction in neurofibromatosis type 1

Disfunção autonçmica termorregulatória na neurofibromatose do tipo 1
Luciana G Madeira
1   Universidade Federal de Minas Gerais, Escola de Educação Física, Fisioterapia e Terapia Ocupacional, Programa de Pós Graduação em Ciências do Esporte, Belo Horizonte MG, Brasil;
,
Renata LF Passos
1   Universidade Federal de Minas Gerais, Escola de Educação Física, Fisioterapia e Terapia Ocupacional, Programa de Pós Graduação em Ciências do Esporte, Belo Horizonte MG, Brasil;
,
Juliana F de Souza
2   Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil.
,
Nilton A Rezende
2   Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil.
,
Luiz O. C. Rodrigues
1   Universidade Federal de Minas Gerais, Escola de Educação Física, Fisioterapia e Terapia Ocupacional, Programa de Pós Graduação em Ciências do Esporte, Belo Horizonte MG, Brasil;
2   Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil.
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ABSTRACT

Objective Neurofibromatosis type 1 (NF1) causes neural and cutaneous disorders and reduced exercise capacity. Exercise/heat exposure increasing internal temperature must be compensated by eccrine sweat function and warmed skin vasodilation. We suspected NF1 could adversely affect eccrine sweat function and/or vascular thermoregulatory responses (VTR).

Methods The eccrine sweat function and VTR of 25 NF1 volunteers (14 males, 11 females; 16–57 years old) were compared with 23 non-NF1 controls matched by sex, age, height and weight (CG). Sweating was induced by 1) pilocarpine 1% iontophoresis (PILO); and 2) by passive heating (HEAT) via the lower third of the legs being immersed in 42°C water for one hour. Previously established eccrine sweat function and VTR protocols were used.

Results The NF1 group showed: a) lower sweat rate than the CG group during PILO; b) a smaller diastolic pressure decrease; and c) higher tympanic temperatures than controls during HEAT (p < 0.05).

Conclusion Reduced sweating and vascular thermoregulatory responses suggest autonomic dysfunction in NF1 individuals.

RESUMO

Objetivo Neurofibromatose do tipo 1 (NF1) causa problemas neurais e cutâneos e diminuição da capacidade física. O aumento da temperatura interna durante exercício e exposição ao calor precisa ser compensada pela função sudorípara écrina (FSE) e aquecimento cutâneo por vasodilatação (RVT). Suspeitou-se clinicamente que a NF1 poderia prejudicar a FSE e a RVT.

Métodos A FSE e RVT de 25 voluntários com NF1 (14 homens, 11 mulheres; 16–57 anos) e de 23 sem-NF1, emparelhados por sexo, idade, estatura e peso corporal, foram medidas com protocolos validados anteriormente. A sudorese foi induzida por iontoforese com pilocarpina (PILO) e aquecimento passivo por imersão das pernas em água a 42°C durante uma hora (HEAT).

Resultados O grupo NF1 apresentou menor taxa de sudorese na situação PILO, menor redução da pressão diastólica e maior temperatura timpânica na situação HEAT (p < 0.05).

Conclusão As respostas sudorípara e vascular reduzidas sugerem disfunção autonçmica nas pessoas com NF1.

neurofibromatosis 1 - sweating - primary dysautonomias - body temperature regulation
neurofibromatose 1 - sudorese - disautonomia primária - regulação da temperatura corporal

Support:

CNPq, FAPEMIG, CAPES, UFMG




Publication History

Received: 27 April 2016

Accepted: 20 June 2016

Article published online:
06 September 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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