Skin sympathetic fiber α-synuclein deposits A potential biomarker for pure autonomic failure
Skin sympathetic fiber α-synuclein deposits A potential biomarker for pure autonomic failure
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DOI：
10.1212/wnl.0b013e3182825127
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发表时间：
2013-02-01
期刊：
影响因子：
9.9
通讯作者：
Liguori, Rocco
中科院分区：
文献类型：
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作者：
Donadio, Vincenzo;Incensi, Alex;Liguori, Rocco
目的：本研究的目的是测试是否外周α-突触核蛋白染色可能是有用的纯自主神经功能衰竭（PAF）的诊断，有助于区分退行性获得性周围自主神经neuropathy.Methods：我们研究了21例慢性周围自主神经病变显示交感神经和副交感神经参与证实的心血管反射和微神经造影术从腓神经。12例患者表现出神经病变的特定原因（获得性自主神经病变），而9例患者没有满足PAF诊断标准的特定获得性原因。15名匹配的健康受试者作为对照。受试者进行皮肤活检从大腿和腿部研究皮肤神经支配和磷酸化的α-突触核蛋白存款在外周axons.Results：躯体和自主神经皮肤神经支配显着减少周围自主神经病变患者相比，对照组。获得性自主神经病变与阵发性房颤之间无差异。在对照组中未发现α-突触核蛋白沉积，但可用于区分获得性和退行性自主神经周围神经病变：所有PAF患者均显示α-突触核蛋白沉积，而获得性自主神经病变患者则不存在。共定位研究揭示了α-突触核蛋白neuritic包涵体在节后交感肾上腺素能和胆碱能神经field.Conclusions：我们的研究表明，寻找神经炎包涵体的磷酸化α-突触核蛋白在皮肤交感神经纤维可以提供一个敏感的在体内生物标志物退行性周围自主神经病变，并可能揭示更多的PAF的发病机制。神经病学（R）2013;80：725-732
Objective: This study aimed to test whether peripheral alpha-synuclein staining might be useful for pure autonomic failure (PAF) diagnosis, helping to differentiate degenerative from acquired peripheral autonomic neuropathy.Methods: We studied 21 patients with chronic peripheral autonomic neuropathy showing sympathetic and parasympathetic involvement as confirmed by cardiovascular reflexes and microneurography from the peroneal nerve. Twelve patients showed a specific cause of neuropathy (acquired autonomic neuropathy) whereas 9 had no specific acquired causes fulfilling the diagnostic criteria for PAF. Fifteen matched healthy subjects served as controls. Subjects underwent skin biopsy from thigh and leg to study skin innervation and phosphorylated alpha-synuclein deposits in the peripheral axons.Results: Somatic and autonomic skin innervations were significantly decreased in patients with peripheral autonomic neuropathy compared to controls. No differences were found between acquired autonomic neuropathy and PAF. The deposits of alpha-synuclein were not found in controls but served to distinguish acquired from degenerative autonomic peripheral neuropathy: all patients with PAF showed alpha-synuclein deposits, which were absent in patients with acquired autonomic neuropathy. Colocalization study disclosed alpha-synuclein neuritic inclusions in the postganglionic sympathetic adrenergic and cholinergic nerve fibers.Conclusions: Our study demonstrated that a search for neuritic inclusions of phosphorylated alpha-synuclein in the skin sympathetic nerve fibers could provide a sensitive in vivo biomarker for degenerative peripheral autonomic neuropathy and may shed more light on the pathogenesis of PAF. Neurology (R) 2013;80:725-732