Small-fiber neuropathy/neuronopathy associated with celiac disease - Skin biopsy findings
Small-fiber neuropathy/neuronopathy associated with celiac disease - Skin biopsy findings
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DOI：
10.1001/archneur.62.10.1574
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发表时间：
2005-10-01
影响因子：
--
通讯作者：
Latov, N
中科院分区：
文献类型：
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作者：
Brannagan, TH;Hays, AP;Latov, N
背景：乳糜泻(CD)在北美日益受到重视，并与周围神经病变相关。目的：报告CD和小纤维神经病患者的临床特征和皮肤活检结果。设计：病例系列。背景：学术周围神经病临床。患者：8例CD和神经病变症状患者。干预：采用三毫米穿孔活检，使用Panaxonal标志物蛋白基因产物9.5评估表皮神经纤维(ENF)密度和无麸质饮食。主要观察指标：临床数据和ENF密度。结果：所有患者都有不对称性麻木和感觉迟钝。3人手部受累比脚更明显，3人面部麻木。5例在神经病变开始后诊断为乳糜泻。血清抗体水平升高：组织转谷氨酰胺酶(n=6)、IgA醇溶蛋白(n=4)和免疫球蛋白醇溶蛋白(n=7)。7例患者神经传导检查正常。一名患者的腓肠肌幅度轻度降低。5例脑神经营养因子密度降低。另有3例患者的脑神经营养因子密度处于正常范围的下限，其中2例有轴突形态改变。3名患者大腿或前臂的脑神经营养因子密度降低，其程度比小腿远端更严重，与非长度依赖的过程相一致。有四人报告称，采用无麸质饮食后情况有所改善。1例术后4个月无改善。2例在接受无麸质饮食时出现症状。结论：皮肤活检结果显示，CD患者可能有涉及细小纤维的神经病变。面部频繁受累，皮肤活检发现非长度依赖的症状模式，提示为感觉神经节病或免疫介导的神经病。一些患者在开始无麸质饮食后症状的改善值得进一步研究。
Background: Celiac disease (CD) is increasingly recognized in North America and is associated with a peripheral neuropathy.Objective: To report the clinical characteristics and skin biopsy results in patients with CD and small-fiber neuropathy symptoms.Design: Case series.Setting: Academic peripheral neuropathy clinic.Patients: Eight patients with CD and neuropathy symptoms.Intervention: Three-millimeter punch biopsy using the panaxonal marker protein gene product 9.5 to assess epidermal nerve fiber (ENF) density and a gluten-free diet.Main Outcome Measure: Clinical data and ENF density.Results: All patients had asymmetric numbness and paresthesias. Three had more prominent involvement of hands than feet, and 3 had facial numbness. Celiac disease was diagnosed in 5 after their neuropathy began. The following serum antibody levels were elevated: tissue transglutaminase (n=6), IgA gliadin (n=4), and IgG gliadin (n = 7). Results of nerve conduction studies were normal in 7 patients. One patient had mildly reduced sural amplitudes. The ENF density was reduced in 5 patients. The ENF density was at the low limit of the normal range in 3 additional patients, 2 of whom had morphologic changes in axons. Three patients had decreased ENF density at the thigh or forearm, which was more severe than at the distal leg, compatible with a non-length-dependent process. Four reported improvement with a gluten-free diet. One had no improvement after 4 months. Symptoms developed in 2 while receiving a gluten-free diet.Conclusions: Patients with CD may have a neuropathy involving small fibers, demonstrated by results of skin biopsy. The pattern of symptoms, with frequent facial involvement and a non-length-dependent pattern on skin biopsy findings, suggests a sensory ganglionopathy or an immune-mediated neuropathy. Improvement of symptoms in some patients after initiating a gluten-free diet warrants further study.