Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia
Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia
复制标题
DOI：
10.1016/s0140-6736(98)05342-2
复制
发表时间：
1998-11-14
期刊：
影响因子：
168.9
通讯作者：
Smith, CML
中科院分区：
文献类型：
--
作者：
Hadjivassiliou, M;Grünewald, R;Smith, CML
背景：共济失调是乳糜泻最常见的神经学表现。一些遗传易感性的个体有麸质敏感的血清学证据，没有明显的胃肠道症状或小肠炎症的证据。这类患者疾病的唯一表现可能是共济失调。我们描述了这种疾病的临床、放射学和神经生理学特征。方法对在英国谢菲尔德皇家哈勒姆郡医院神经内科门诊就诊的共济失调患者进行谷蛋白敏感性筛查，检测麦胶蛋白抗体滴度。其他原因引起共济失调者排除在外。我们进行了临床、神经生理、神经放射学检查，并在两例中进行了神经病理学检查。结果确诊谷蛋白共济失调28例。所有患者均有步态共济失调，大部分患者有肢体共济失调。步态共济失调越严重，病程越长。没有患者有震颤或其他锥体外系特征。19例患者神经生理检查显示有不同形式的周围神经病变。16例患者无胃肠道症状。2例十二指肠远端活检显示淋巴细胞浸润，11例改变与乳糜泻相符。6例患者mri表现为小脑萎缩。对两名死亡的患者进行了尸检；小脑淋巴细胞浸润，脊髓后柱损伤，周围神经稀疏浸润。谷蛋白敏感性是明显特发性共济失调的重要原因，可能是进行性的。共济失调是小脑、脊髓后柱和周围神经受到免疫损伤的结果。我们提出术语麸质共济失调来描述这种疾病。
Background Ataxia is the commonest neurological manifestation of coeliac disease. Some individuals with genetic susceptibility to the disease have serological evidence of gluten sensitivity without overt gastrointestinal symptoms or evidence of small-bower inflammation. The sole manifestation of disease in such patients may be ataxia. We describe the clinical, radiological, and neurophysiological features of this disorder.Methods Patients with ataxia attending the neurology outpatient clinics at the royal Hallamshire Hospital, Sheffield, UK, were screened for gluten sensitivity as shown by the titre of antibody to gliadin. Those with other causes of ataxia were excluded. we carried out clinical, neurophysiological, neuroradiological, and, in two cases, neuropathological examinations.Findings 28 patients with gluten ataxia were identified. All had gait ataxia and most had limb ataxia. Those with more severe gait ataxia had longer disease duration. No patient had tremor or other extrapyramidal features. 19 patients showed some form of peripheral neuropathy on neurophysiological examination. 16 patients had no gastrointestinal symptoms. Distal duodenal biopsy showed lymphocytic infiltration in two patients, and changes compatible with coeliac disease in 11. Six patients had evidence of cerebellar atrophy on magnetic-resonance imaging. Necropsy was done on two patients who died; there was lymphocytic infiltration of the cerebellum, damage to the posterior columns of the spinal cord, and sparse infiltration of the peripheral nerves.Interpretation Gluten sensitivity is an important cause of apparently idiopathic ataxia and may be progressive. The ataxia is a result of immunological damage to the cerebellum, to the posterior columns of the spinal cord, and to peripheral nerves. We propose the term gluten ataxia to describe this disorder.