Hadjivassiliou M, Boscolo S, Davies-Jones G A B, Grünewald R A, Not T, Sanders D S, Simpson J E, Tongiorgi E, Williamson C A, Woodroofe N M
Department of Clinical Neurology, The Royal Hallamshire Hospital, Sheffield, UK.
Neurology. 2002 Apr 23;58(8):1221-6. doi: 10.1212/wnl.58.8.1221.
To characterize humoral response to cerebellum in patients with gluten ataxia.
Gluten ataxia is a common neurologic manifestation of gluten sensitivity.
The authors assessed the reactivity of sera from patients with gluten ataxia (13), newly diagnosed patients with celiac disease without neurologic dysfunction (24), patients with other causes of cerebellar degeneration (11), and healthy control subjects (17) using indirect immunocytochemistry on human cerebellar and rat CNS tissue. Cross-reactivity of a commercial IgG antigliadin antibody with human cerebellar tissue also was studied.
Sera from 12 of 13 patients with gluten ataxia stained Purkinje cells strongly. Less intense staining was seen in some but not all sera from patients with newly diagnosed celiac disease without neurologic dysfunction. At high dilutions (1:800) staining was seen only with sera from patients with gluten ataxia but not in control subjects. Sera from patients with gluten ataxia also stained some brainstem and cortical neurons in rat CNS tissue. Commercial anti-gliadin antibody stained human Purkinje cells in a similar manner. Adsorption of the antigliadin antibodies using crude gliadin abolished the staining in patients with celiac disease without neurologic dysfunction, but not in those with gluten ataxia.
Patients with gluten ataxia have antibodies against Purkinje cells. Antigliadin antibodies cross-react with epitopes on Purkinje cells.
描述麸质共济失调患者对小脑的体液免疫反应。
麸质共济失调是麸质敏感性常见的神经系统表现。
作者使用人小脑和大鼠中枢神经系统组织的间接免疫细胞化学方法,评估了麸质共济失调患者(13例)、新诊断的无神经功能障碍的乳糜泻患者(24例)、其他小脑变性原因患者(11例)和健康对照者(17例)血清的反应性。还研究了商业IgG抗麦醇溶蛋白抗体与人小脑组织的交叉反应性。
13例麸质共济失调患者中的12例血清对浦肯野细胞有强烈染色。在一些但并非所有新诊断的无神经功能障碍的乳糜泻患者血清中可见较弱的染色。在高稀释度(1:800)时,仅麸质共济失调患者的血清出现染色,而对照者血清未出现。麸质共济失调患者的血清在大鼠中枢神经系统组织中也对一些脑干和皮质神经元进行了染色。商业抗麦醇溶蛋白抗体以类似方式对人浦肯野细胞进行了染色。使用粗制麦醇溶蛋白吸附抗麦醇溶蛋白抗体消除了无神经功能障碍的乳糜泻患者的染色,但未消除麸质共济失调患者的染色。
麸质共济失调患者有针对浦肯野细胞的抗体。抗麦醇溶蛋白抗体与浦肯野细胞上的表位发生交叉反应。