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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282XOn-line version ISSN 1678-4227

Abstract

MADEIRA, Luciana G et al. Autonomic thermoregulatory dysfunction in neurofibromatosis type 1. Arq. Neuro-Psiquiatr. [online]. 2016, vol.74, n.10, pp.796-802. ISSN 0004-282X.  http://dx.doi.org/10.1590/0004-282X20160122.

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.

Keywords : neurofibromatosis 1; sweating; primary dysautonomias; body temperature regulation.

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