Lewy body pathology involves cutaneous nerves
Lewy body pathology involves cutaneous nerves
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DOI：
10.1097/nen.0b013e318186de48
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发表时间：
2008-10-01
影响因子：
3.2
通讯作者：
Murayama, Shigeo
中科院分区：
文献类型：
--
作者：
Ikemura, Masako;Saito, Yuko;Murayama, Shigeo
周围自主神经系统受累是路易体（LB）疾病的核心特征，包括帕金森病（PD）、PD伴痴呆和LB伴痴呆。为了研究皮肤活检在 LB 疾病诊断中的潜在用途，我们评估了 279 名连续尸检患者的腹壁和上臂屈肌表面皮肤样本中周围神经的抗磷酸化 α-突触核蛋白免疫反应性，这些患者的数据在 2002 年至 2005 年间在衰老研究脑库中登记。20 名患者的真皮无髓鞘纤维中显示出阳性免疫反应性。 85 名中枢神经系统和肾上腺有 LB 病理学的患者，后者代表周围自主神经系统交感神经节的替代；在 194 名无 CNS LB 病理的患者中未观察到反应性。在 1995 年以来对 142 名患有亚临床或临床 LB 疾病的尸检患者进行回顾性研究中，皮肤免疫反应阳性的敏感性在 PD 和 PD 伴痴呆中为 70%，在伴 LB 痴呆中为 40%。在多系统萎缩、进行性核麻痹和皮质基底节变性的情况下，皮肤免疫反应性不存在。我们首次证明皮肤参与其中，并且可能是LB疾病病理诊断的高度特异性和有用的活检部位。
Involvement of the peripheral autonomic nervous system is a core feature of Lewy body (LB) diseases, including Parkinson disease (PD), PD with dementia, and dementia with LBs. To investigate the potential use of skin biopsy for the diagnosis of LB diseases, we assessed anti-phosphorylated alpha-synuclein immunoreactivity in peripheral nerves in samples of skin from the abdominal wall and flexor surface of the upper arm in 279 prospectively studied consecutively autopsied patients whose data were registered at the Brain Bank for Aging Research between 2002 and 2005. Positive immunoreactivity was demonstrated in the unmyelinated fibers of the dermis in 20 of 85 patients with LB pathology in the CNS and the adrenal glands, the latter representing a substitute for peripheral autonomic nervous system sympathetic ganglia; no reactivity was seen in 194 patients without CNS LB pathology. In 142 retrospectively Studied patients autopsied from 1995 onward who had subclinical or clinical LB disease, the sensitivity of the positive skin immunoreactivity was 70% in PD and PD with dementia and 40% in dementia with LBs. Skin immunoreactivity was absent in cases of multiple-system atrophy, progressive nuclear palsy, and corticobasal degeneration. We demonstrate for the first time that the skin is involved and may be a highly specific and useful biopsy site for the pathological diagnosis of LB diseases.