Anheim M, Degos B, Echaniz-Laguna A, Fleury M, Grucker M, Tranchant C
Service des Maladies du Système Nerveux et du Muscle, Clinique Neurologique, Hôpital Civil de Strasbourg, Strasbourg.
Rev Neurol (Paris). 2006 Feb;162(2):214-21. doi: 10.1016/s0035-3787(06)75002-0.
Gluten ataxia refers to the association of idiopathic ataxia despite exhaustive investigations with gluten sensitivity defined by anti-gliadin antibodies (AGA) presence in blood. This is a controversial concept.
We screened 33 patients, who were hospitalized in 2003 and had subacute or chronic ataxia for presence of circulating AGA. Twelve patients were positive and their clinical and biological features were studied.
Among the twelve patients, we concluded that gluten ataxia was present in only eight, including one case of celiac disease. Among these eight patients, five had the usual features of gluten ataxia (progressive cerebellar ataxia affecting mainly lower limbs), but one patient presented unusual left cerebellar hemisyndrome and the two others displayed polyneuropathy with proprioceptive ataxia. Cerebellar atrophy was confirmed with magnetic resonance imaging in five cases and association with other antibodies was found in six cases. Among the four other patients positive for AGA, investigations revealed one case of multiple sclerosis, one case of late-onset Friedreich ataxia, one case of basilar tuberculous meningitis and one case of type 2 diabetes.
Screening for AGA presence should be systematically performed at presentation of patients with unknown etiology ataxia; in the event AGA are present without any other etiology, treatment with gluten-free diet must be discussed. However, the responsibility of AGA in the pathogenesis of neurological signs is highly debatable and further experimental work is required. Two pathophysiological hypotheses are suggested: (1) overexpression of cerebellar epitopes, in case of primary cerebellar pathology, leading to excessive immune response directed against these epitopes; and (2) molecular mimicry with cross-reactivity of antigens usually directed against gliadin, but also recognizing Purkinje cells epitopes.
麸质共济失调是指尽管经过详尽检查仍为特发性共济失调，且血液中存在抗麦醇溶蛋白抗体（AGA）所定义的麸质敏感性。这是一个存在争议的概念。
我们对2003年住院的33例患有亚急性或慢性共济失调的患者进行筛查，以检测循环AGA的存在。12例患者呈阳性，并对其临床和生物学特征进行了研究。
在这12例患者中，我们得出结论，仅8例存在麸质共济失调，其中包括1例乳糜泻。在这8例患者中，5例具有麸质共济失调的常见特征（进行性小脑共济失调，主要影响下肢），但1例患者表现为不常见的左侧小脑半综合征，另外2例表现为伴有本体感觉性共济失调的多发性神经病。5例通过磁共振成像证实存在小脑萎缩，6例发现与其他抗体有关联。在另外4例AGA阳性的患者中，检查发现1例多发性硬化症、1例迟发性弗里德赖希共济失调、1例基底结核性脑膜炎和1例2型糖尿病。
对于病因不明的共济失调患者，应系统地筛查是否存在AGA；如果存在AGA且无其他病因，必须讨论采用无麸质饮食进行治疗。然而，AGA在神经体征发病机制中的作用极具争议，需要进一步的实验研究。提出了两种病理生理假说：（1）在原发性小脑病变的情况下，小脑表位过度表达，导致针对这些表位的过度免疫反应；（2）分子模拟，抗原通常针对麦醇溶蛋白发生交叉反应，但也识别浦肯野细胞表位。
