Department of Neurology, University of Marburg, Marburg, Germany.
Mov Disord. 2009 Dec 15;24(16):2358-62. doi: 10.1002/mds.22821.
In celiac disease (CD), the gut is the typical manifestation site but atypical neurological presentations are thought to occur in 6 to 10% with cerebellar ataxia being the most frequent symptom. Most studies in this field are focused on patients under primary neurological care. To exclude such an observation bias, patients with biopsy proven celiac disease were screened for neurological disease. A total of 72 patients with biopsy proven celiac disease (CD) (mean age 51 +/- 15 years, mean disease duration 8 +/- 11 years) were recruited through advertisements. All participants adhered to a gluten-free diet. Patients were interviewed following a standard questionnaire and examined clinically for neurological symptoms. Medical history revealed neurological disorders such as migraine (28%), carpal tunnel syndrome (20%), vestibular dysfunction (8%), seizures (6%), and myelitis (3%). Interestingly, 35% of patients with CD reported of a history of psychiatric disease including depression, personality changes, or even psychosis. Physical examination yielded stance and gait problems in about one third of patients that could be attributed to afferent ataxia in 26%, vestibular dysfunction in 6%, and cerebellar ataxia in 6%. Other motor features such as basal ganglia symptoms, pyramidal tract signs, tics, and myoclonus were infrequent. 35% of patients with CD showed deep sensory loss and reduced ankle reflexes in 14%. Gait disturbances in CD do not only result from cerebellar ataxia but also from proprioceptive or vestibular impairment. Neurological problems may even develop despite strict adherence to a gluten-free diet.
在乳糜泻(CD)中,肠道是典型的表现部位,但据认为不典型的神经表现发生在 6%至 10%的患者中,小脑性共济失调是最常见的症状。该领域的大多数研究都集中在接受初级神经护理的患者上。为了排除这种观察偏见,对经活检证实患有乳糜泻的患者进行了神经疾病筛查。通过广告招募了 72 名经活检证实患有乳糜泻(CD)的患者(平均年龄 51 +/- 15 岁,平均病程 8 +/- 11 年)。所有参与者均遵循无麸质饮食。通过标准问卷对患者进行访谈并进行临床神经症状检查。病史显示存在神经疾病,如偏头痛(28%)、腕管综合征(20%)、前庭功能障碍(8%)、癫痫发作(6%)和脊髓炎(3%)。有趣的是,35%的 CD 患者报告有精神疾病史,包括抑郁、人格改变,甚至精神病。体格检查发现约三分之一的患者存在姿势和步态问题,其中 26%可归因于感觉传入性共济失调,6%可归因于前庭功能障碍,6%可归因于小脑性共济失调。其他运动特征,如基底节症状、锥体束征、抽搐和肌阵挛则较为少见。35%的 CD 患者出现深感觉丧失和 14%的踝反射减弱。尽管严格遵循无麸质饮食,CD 患者的步态障碍也不仅由小脑性共济失调引起,还可由本体感觉或前庭功能障碍引起。即使严格遵循无麸质饮食,也可能会出现神经问题。