Sato Kenji, Nanri Kazunori
Department of Neurology, Yokufukai Hospital.
Brain Nerve. 2017 Aug;69(8):933-940. doi: 10.11477/mf.1416200845.
Gluten-related disorders (GRDs) are conditions that develop in response to the common trigger of gluten ingestion and manifest as a variety of clinical symptoms. GRDs have been considered rare in Asian countries, including Japan, because of lower consumption of wheat products than in Europe and the U.S.A. and differences in genetic background. Recently, however, GRDs, such as celiac disease and gluten ataxia, have been reported in Japan, albeit sporadically and their presence is now recognized in this country. Gluten ataxia is defined as an anti-gliadin antibody positive sporadic ataxia. Recently, it was reported that the presence of anti-transglutaminase-6 (TG6) antibody can be used to diagnose gluten ataxia. Herein, we will review evidence relating to gluten ataxia and report two cases of anti-TG6 antibody positive gluten ataxia. In patients with gluten ataxia, sensory disturbance is generally considered to be so mild that it contributes minimally to ataxia. However, our patients showed a positive Romberg sign. Deep sensory disturbance, in addition to cerebellar disturbance, may have been involved in the clinical symptoms of our cases.
麸质相关疾病（GRDs）是因摄入麸质这一常见诱因引发的病症，表现为多种临床症状。由于亚洲国家（包括日本）的小麦制品消费量低于欧美国家，且存在基因背景差异，GRDs在这些国家曾被认为较为罕见。然而，近年来，日本已报告了一些GRDs病例，如乳糜泻和麸质共济失调，尽管较为零散，但该国目前已认识到它们的存在。麸质共济失调被定义为抗麦醇溶蛋白抗体阳性的散发性共济失调。最近有报道称，抗转谷氨酰胺酶6（TG6）抗体的存在可用于诊断麸质共济失调。在此，我们将回顾与麸质共济失调相关的证据，并报告两例抗TG6抗体阳性的麸质共济失调病例。在麸质共济失调患者中，感觉障碍通常被认为很轻微，对共济失调的影响极小。然而，我们的患者出现了阳性罗姆伯格征。除小脑功能障碍外，深部感觉障碍可能也与我们病例的临床症状有关。
