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.
Donadio
Vincenzo
V
Department of Neurological Sciences, University of Bologna, Via U. Foscolo 7, Bologna, Italy. donadio@neuro.unibo.it
Nolano
Maria
M
Elam
Mikael
M
Montagna
Pasquale
P
Provitera
Vincenzo
V
Bugiardini
Enrico
E
Baruzzi
Agostino
A
Santoro
Lucio
L
Liguori
Rocco
R
D000368:Aged; D001338:Autonomic Fibers, Postganglionic / Q000503:physiopathology; D001342:Autonomic Nervous System Diseases / Q000503:physiopathology; D006801:Humans; D007007:Hypohidrosis / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000503:physiopathology*; D017779:Sympathetic Fibers, Postganglionic / Q000503:physiopathology; D013564:Sympathetic Nervous System / Q000503:physiopathology