Autonomic thermoregulatory dysfunction in neurofibromatosis type 1

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...

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Main Authors: Luciana G Madeira, Renata LF Passos, Juliana F de Souza, Nilton A Rezende, Luiz O. C. Rodrigues
Format: Article
Language:English
Published: Thieme Revinter Publicações
Series:Arquivos de Neuro-Psiquiatria
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2016001000796&lng=en&tlng=en
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author Luciana G Madeira
Renata LF Passos
Juliana F de Souza
Nilton A Rezende
Luiz O. C. Rodrigues
author_facet Luciana G Madeira
Renata LF Passos
Juliana F de Souza
Nilton A Rezende
Luiz O. C. Rodrigues
author_sort Luciana G Madeira
collection DOAJ
description 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.
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series Arquivos de Neuro-Psiquiatria
spelling doaj-art-5b6fd0c8ef5449af85576b515bd0300e2025-08-20T03:26:30ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria1678-4227741079680210.1590/0004-282X20160122S0004-282X2016001000796Autonomic thermoregulatory dysfunction in neurofibromatosis type 1Luciana G MadeiraRenata LF PassosJuliana F de SouzaNilton A RezendeLuiz O. C. RodriguesABSTRACT 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2016001000796&lng=en&tlng=enneurofibromatosis 1sweatingprimary dysautonomiasbody temperature regulation
spellingShingle Luciana G Madeira
Renata LF Passos
Juliana F de Souza
Nilton A Rezende
Luiz O. C. Rodrigues
Autonomic thermoregulatory dysfunction in neurofibromatosis type 1
Arquivos de Neuro-Psiquiatria
neurofibromatosis 1
sweating
primary dysautonomias
body temperature regulation
title Autonomic thermoregulatory dysfunction in neurofibromatosis type 1
title_full Autonomic thermoregulatory dysfunction in neurofibromatosis type 1
title_fullStr Autonomic thermoregulatory dysfunction in neurofibromatosis type 1
title_full_unstemmed Autonomic thermoregulatory dysfunction in neurofibromatosis type 1
title_short Autonomic thermoregulatory dysfunction in neurofibromatosis type 1
title_sort autonomic thermoregulatory dysfunction in neurofibromatosis type 1
topic neurofibromatosis 1
sweating
primary dysautonomias
body temperature regulation
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2016001000796&lng=en&tlng=en
work_keys_str_mv AT lucianagmadeira autonomicthermoregulatorydysfunctioninneurofibromatosistype1
AT renatalfpassos autonomicthermoregulatorydysfunctioninneurofibromatosistype1
AT julianafdesouza autonomicthermoregulatorydysfunctioninneurofibromatosistype1
AT niltonarezende autonomicthermoregulatorydysfunctioninneurofibromatosistype1
AT luizocrodrigues autonomicthermoregulatorydysfunctioninneurofibromatosistype1