Autonomic innervation in multiple system atrophy and pure autonomic failure
Autonomic innervation in multiple system atrophy and pure autonomic failure
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DOI：
10.1136/jnnp.2009.198135
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发表时间：
2010-12-01
影响因子：
11
通讯作者：
Liguori, R.
中科院分区：
文献类型：
--
作者：
Donadio, V.;Cortelli, P.;Liguori, R.
背景单纯自主神经衰竭（PAF）和多系统萎缩（MSA）都以慢性自主神经功能障碍为特征，尽管表现出不同的残疾和预后。皮肤自主神经功能的间接测试评价揭示了这些疾病的相互矛盾的结果。在这里，作者报告的第一个直接分析皮肤交感神经纤维，包括结构和功能的PAF和MSA，以确定不同的基础自主神经病变的网站，这可能有助于区分两个conditions.Methods作者研究了8例患者可能MSA（平均年龄60 +/- 5岁）和9例患者符合诊断标准的PAF（64 +/- 8岁）。他们接受了头高位倾斜试验（HUTT），广泛的显微神经摄影检查腓神经的肌肉和皮肤交感神经活动，并从手指上取下皮肤活检，结果MSA和PAF患者在HUTT时表现出相似的神经源性直立性低血压，显微神经摄影试验记录交感神经活动，表明慢性自主神经功能障碍的相似程度。相反，他们提出了不同的皮肤自主神经支配的免疫荧光分析。MSA患者表现出普遍保留的皮肤自主神经支配与显着高于PAF患者表现出显着的节后交感神经支配。在MSA患者病程长，形态学异常和/或自主神经评分略有下降，可以发现在腿反映轻度postganglioninvolved.Conclusion自主神经支配的皮肤附件的研究是一种可靠的方法，可以帮助MSA和PAF的鉴别。
Background Pure autonomic failure (PAF) and multiple system atrophy (MSA) are both characterised by chronic dysautonomia although presenting different disability and prognosis. Skin autonomic function evaluation by indirect tests has revealed conflicting results in these disorders. Here, the authors report the first direct analysis of skin sympathetic fibres including structure and function in PAF and MSA to ascertain different underlying autonomic lesion sites which may help differentiate between the two conditions.Methods The authors studied eight patients with probable MSA (mean age 60 +/- 5 years) and nine patients fulfilling diagnostic criteria for PAF (64 +/- 8 years). They underwent head-up tilt test (HUTT), extensive microneurographic search for muscle and skin sympathetic nerve activities from peroneal nerve and punch skin biopsies from finger, thigh and leg to evaluate cholinergic and adrenergic autonomic dermal annexes innervation graded by a semiquantitative score presenting a high level of reliability.Results MSA and PAF patients presented a comparable neurogenic orthostatic hypotension during HUTT and high failure rate of microneurographic trials to record sympathetic nerve activity, suggesting a similar extent of chronic dysautonomia. In contrast, they presented different skin autonomic innervation in the immunofluorescence analysis. MSA patients showed a generally preserved skin autonomic innervation with a significantly higher score than PAF patients showing a marked postganglionic sympathetic denervation. In MSA patients with a long disease duration, morphological abnormalities and/or a slightly decreased autonomic score could be found in the leg reflecting a mild postganglionic involvement.Conclusion Autonomic innervation study of skin annexes is a reliable method which may help differentiate MSA from PAF.