Objective

Gluten neuropathy (GN) is the term used to describe peripheral neuropathy that occurs in patients with gluten sensitivity (GS) or coeliac disease (CD) in the absence of other risk factors. We aimed to describe the neurophysiological progression rate of GN across time and look into the potential role of genetic susceptibility in its development.

Methods

This is a cohort study of 45 patients with GN with a mean follow-up period of 8 ± 5 years. The assessments included clinical and neurophysiological data and HLA-DQ genotyping.

Results

The mean age at diagnosis was 60 ± 12 years. The majority of patients had a length-dependent neuropathy (75.6%), whereas the rest were diagnosed with sensory ganglionopathy (SG).

DQA1*02-positive patients were more likely to suffer with SG compared to the DQA1*02 negative patients (60% versus 13.8%, p = 0.009). There was also a trend for statistical significance regarding the DQB1*06 allele and the DQA1*01/DQB1*06 haplotype were found more frequently in patients with GN than in healthy controls (p = 0.026 and p = 0.047, respectively). A linear effect of time on the neurophysiological findings was found in radial sensory nerve action potential (1.9% mean annual decrement, p = 0.036), sural sensory nerve action potential (3.3% mean annual decrement, p = 0.013) and tibial nerve motor compound action potential (6.5% mean annual decrement, p < 0.001) amplitudes, independently from age or gender.

Conclusions

GN is a late manifestation of GS and CD. The majority of patients have the length-dependent neuropathy with a linear deterioration over time. HLA genotyping of GS and CD patients who suffer from neuropathic symptoms is recommended as it can help identifying patients at risk for developing SG.

中文翻译：

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面筋神经病：电生理进展和HLA关联。

目的

面筋神经病（GN）是一个术语，用于描述在没有其他危险因素的情况下发生于面筋敏感性（GS）或腹腔疾病（CD）的患者中发生的周围神经病。我们旨在描述跨时间的GN的神经生理学进展速率，并探讨遗传易感性在其发展中的潜在作用。

方法

这是一项针对45例GN患者的队列研究，平均随访期为8±5年。评估包括临床和神经生理学数据以及HLA-DQ基因分型。

结果

诊断时的平均年龄为60±12岁。大多数患者有长度依赖性神经病（75.6％），而其余患者被诊断为感觉神经节病（SG）。

与DQA1 * 02阴性患者相比，DQA1 * 02阳性患者更有可能患有SG（60％比13.8％，p  = 0.009）。与健康对照组相比，GN患者中DQB1 * 06等位基因和DQA1 * 01 / DQB1 * 06单倍型也有统计学意义的趋势（分别为p  = 0.026和p  = 0.047）。时间对神经生理学结果的线性影响存在于radial神经感觉神经动作电位（1.9％年平均下降，p  = 0.036），腓肠感觉神经动作电位（3.3％年平均下降，p  = 0.013）和胫神经运动复合物潜在动作（平均每年减少6.5％，p <0.001）的振幅，与年龄或性别无关。

结论

GN是GS和CD的晚期表现。大多数患者患有长度依赖性神经病，并随时间呈线性恶化。建议对患有神经病性症状的GS和CD患者进行HLA基因分型，因为它可以帮助识别有发生SG风险的患者。