Epidermal nerve fiber density in sensory ganglionopathies: Clinical and neurophysiologic correlations

Epidermal nerve fiber density in sensory ganglionopathies: Clinical and neurophysiologic correlations
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DOI:
10.1002/mus.1107
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发表时间:
2001-08-01
期刊:
影响因子:
3.4
通讯作者:
Pareyson, D
Pareyson, D
中科院分区:
医学3区
文献类型:
--
作者:
Lauria, G;Sghirlanzoni, A;Pareyson, D

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我们通过皮肤活检和定量感觉测试(QST)评估了体细胞无髓鞘纤维在感觉神经节病变中的作用。16例神经节病变患者、16例轴突神经病变患者和15例正常人分别在大腿近端和远端进行皮肤活检。采用抗蛋白基因产物9.5对表皮内神经纤维(IENF)进行免疫染色,光镜下定量测定其线密度。神经纤维和基底膜双染色的共聚焦显微镜研究也进行了。健康受试者和神经病患者的IENF密度呈典型的近端到远端梯度;在神经病变中,小腿远端值明显较低,证实了皮肤神经支配的长度依赖性丧失。相反,有痛觉过敏症状的神经节病患者在大腿近端和远端之间没有表现出任何IENF密度的变化。感觉神经节病中表皮去神经支配的独特模式反映了躯体无髓鞘纤维的变性,这种变性不依赖于长度,这与临床和神经生理学观察一致,并支持诊断。(C) 2001约翰威利父子公司
We assessed the involvement of somatic unmyelinated fibers in sensory ganglionopathies by skin biopsy and quantitative sensory testing (QST). Sixteen patients with ganglionopathy, 16 with axonal neuropathy, and 15 normal controls underwent skin biopsy at the proximal thigh and the distal leg. Intraepidermal nerve fibers (IENF) were immunostained by anti-protein gene product 9.5, and their linear density was quantified under light microscopy. Confocal microscopy studies with double staining of nerve fibers and basement membrane were also performed. Healthy subjects and neuropathy patients showed the typical proximodistal gradient of IENF density; in neuropathies, values were significantly lower at the distal site of the leg, confirming the length-dependent loss of cutaneous innervation. Conversely, ganglionopathy patients with hyperalgesic symptoms did not show any change of IENF density between the proximal thigh and the distal leg. The distinct pattern of epidermal denervation seen in sensory ganglionopathy reflected the degeneration of somatic unmyelinated fibers in a fashion that was not length-dependent, which was consistent with both clinical and neurophysiologic observations and supported the diagnosis. (C) 2001 John Wiley & Sons, Inc.