Anhidrosis in multiple system atrophy: a preganglionic sudomotor dysfunction?

• Anhidrosis occurs in the majority of multiple system atrophy (MSA) patients but the underlying site of lesion is not well established. We describe three patients with long-standing MSA and anhidrosis diagnosed on the basis of a thermoregulatory sweating test. In biopsies of anhidrotic skin, immunofluorescence analysis disclosed a well preserved postganglionic sudomotor innervation in all three patients supporting the hypothesis of a preganglionic nerve fiber lesion underlying their anhidrosis. Postganglionic sudomotor fiber integrity was also confirmed by normal electrodermal responses in one patient, whereas such responses and microneurographically detectable skin sympathetic nerve activity were absent in the other two MSA patients, suggesting a functional inactivity of structurally intact postganglionic sympathetic skin fibers.

Nyckelord

Aged

Autonomic Fibers

Postganglionic

physiopathology

Autonomic Nervous System Diseases

physiopathology

Humans

Hypohidrosis

etiology

Male

Middle Aged

Multiple System Atrophy

physiopathology

Sympathetic Fibers

Postganglionic

physiopathology

Sympathetic Nervous System

physiopathology

Publikations- och innehållstyp

ref (ämneskategori)

art (ämneskategori)