Autonomic thermoregulatory dysfunction in neurofibromatosis type 1

作者：Madeira Luciana G; Passos Renata L F; de Souza Juliana F; Rezende Nilton A; Rodrigues Luiz O C^*

来源：Arquivos de Neuro-Psiquiatria, 2016, 74(10): 796-802.

DOI：10.1590/0004-282X20160122

• neurofibromatosis 1
• sweating
• primary dysautonomias
• body temperature regulation

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 degrees 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.

• 出版日期2016-10