Anhidrosis in multiple system atrophy: A preganglionic sudomotor dysfunction?

被引:22
作者
Donadio, Vincenzo [1 ]
Nolano, Maria [2 ]
Elam, Mikael [3 ]
Montagna, Pasquale [1 ]
Provitera, Vincenzo [2 ]
Bugiardini, Enrico [1 ]
Baruzzi, Agostino [1 ]
Santoro, Lucio [4 ]
Liguori, Rocco [1 ]
机构
[1] Univ Bologna, Dipartimento Sci Neurol, I-40123 Bologna, Italy
[2] IRCCS, Dept Neurol, Salvatore Maugeri Fdn, Telese Terme, BN, Italy
[3] Univ Gothenburg, Sahlgrens Univ Hosp, Dept Clin Neurophysiol, Gothenburg, Sweden
[4] Univ Naples Federico 2, Dept Neurol, Naples, Italy
关键词
multiple system atrophy; anhidrosis; skin biopsy; microneurography; preganglionic sudomotor lesion;
D O I
10.1002/mds.21972
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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. (c) 2008 Movement Disorder Society.
引用
收藏
页码:885 / 888
页数:4
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