Glutamic acid decarboxylase autoimmunity with brainstem, extrapyramidal, and spinal cord dysfunction
Glutamic acid decarboxylase autoimmunity with brainstem, extrapyramidal, and spinal cord dysfunction
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DOI：
10.4065/81.9.1207
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发表时间：
2006-09-01
影响因子：
8.9
通讯作者：
Lennon, Vanda A.
中科院分区：
文献类型：
--
作者：
Pittock, Sean J.;Yoshikawa, Hiroaki;Lennon, Vanda A.
目的：描述与谷氨酸脱羧酶（GAD 65）autoimmunity.Patients和方法：本回顾性研究（1987-2003年）描述了62例偶然发现有选择性结合到突触丰富的中枢神经系统组织的血清自身抗体。通过放射免疫沉淀测定确定免疫染色模式为GAD 65特异性的。这些病例是在提交用于使用间接免疫荧光进行副肿瘤自身抗体评价的样本中确定的。在任何情况下，GAD 65或任何其他胰岛细胞抗体testing been requested.RESULTS：在大多数情况下，患者的介绍最初被认为是神经退行性或炎症（多发性硬化症或副肿瘤）。发病时的中位年龄为50岁，77%为女性。在马约诊所就诊的44名患者中，23%是非洲裔美国人;相比之下，马约诊所的神经科患者中非洲裔美国人不到10%。中位随访时间为24个月。GAD 65抗体的放射免疫沉淀测定值极高（中位数，1429 nmol/L;四分位数间距，643-3078 nmol/L），并与免疫荧光滴度显著相关（中位数，3840;四分位数间距，1920- 15，360; r=0.81; P 20 nmol/L），值得考虑免疫治疗。
OBJECTIVE: To describe novel neurological manifestations associated with glutamic acid decarboxylase (GAD65) autoimmunity.PATIENTS AND METHODS: This retrospective study (1987-2003) describes 62 patients incidentally found to have a serum autoantibody that bound selectively to synapse-rich central nervous system tissues. The immunostaining pattern was determined to be GAD65-specific by radioimmunoprecipitation assay. These cases were identified among samples submitted for paraneoplastic autoantibody evaluation using indirect immunofluorescence. In no case had GAD65 or any other islet cell antibody testing been requested.RESULTS: In most cases, the patients' presentations were initially considered neurodegenerative or inflammatory (multiple sclerosis or paraneoplastic). Median age at onset was 50 years, and 77% were women. Of the 44 patients seen at the Mayo Clinic, 23% were African American; in contrast, less than 10% of Mayo Clinic's neurology patients are African American. Median follow-up was 24 months. The radloimmunoprecipitation assay values for GAD65 antibody were extremely high (median, 1429 nmol/L; interquartille range, 643-3078 nmol/L) and correlated significantly with immunofluorescence titers (median, 3840; interquartile range, 1920-15,360; r=0.81; P 20 nmol/L), merits consideration of immunotherapy.