Autonomic thermoregulatory dysfunction in neurofibromatosis type 1

Luciana G Madeira; Renata LF Passos; Juliana F de Souza; Nilton A Rezende; Luiz O. C. Rodrigues

doi:10.1590/0004-282X20160122

Arquivos de Neuro-Psiquiatria, Table of Contents

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

¹Universidade 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

¹Universidade 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

²Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil.

,

Nilton A Rezende

²Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil.

,

Luiz O. C. Rodrigues

¹Universidade Federal de Minas Gerais, Escola de Educação Física, Fisioterapia e Terapia Ocupacional, Programa de Pós Graduação em Ciências do Esporte, Belo Horizonte MG, Brasil;

²Universidade Federal de Minas Gerais, Hospital das Clínicas, Centro de Referência em Neurofibromatoses, Belo Horizonte MG, Brasil.

› Author Affiliations

Abstract

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

Full Text

References

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