AUTOIMMUNE ASPECTS OF DISEASE

僵硬人综合征是一种罕见的自身免疫性神经系统疾病， 被认为是由GABA能神经元损伤引起的。 一个突出 针对GABA合成酶的体液免疫应答， 谷氨酸脱羧酶（GAD），存在于大多数个体中 受僵人综合症影响 通过免疫组织化学，我们研究了 3例患者血清中存在GAD抗体， 脑脊液1例。 将各种稀释液置于 福尔马林固定的大鼠大脑切片。 一种荧光图案， 在大脑的许多区域都显示出与 当使用绵羊抗GAD抗血清时观察到。 脑脊液 也表明GAD自身抗体的存在，虽然在较低的 滴度 这些结果支持了僵硬人综合征是一种 攻击中枢神经系统GABA能神经元的自身免疫性疾病。 我们计划 未来的研究将尝试使用“僵硬人”建立一个动物模型， 血清的

Stiff-Man Syndrome is a rare autoimmune neurological disease that is thought to result from an impairment of GABAergic neurons. A prominent humoral immune response directed against the GABA-synthesizing enzyme, glutamic acid decarboxylase (GAD), occurs in the majority of individuals affected by Stiff-Man Syndrome. By immunohistochemistry we investigated the presence of GAD antibodies in the serum of 3 patients and the cerebrospinal fluid of 1 patient. A variety of dilutions were placed on sections of formalin fixed rat brains. A pattern of fluorescence was revealed in numerous regions of the brain that was identical to that observed when sheep antiserum to GAD was employed. The cerebrospinal fluid also indicated the presence of GAD autoantibodies, although at a lower titer. These results support the notion that the Stiff-Man Syndrome is an autoimmune disease that attacks GABAergic neurons of the CNS. We plan future studies to attempt to create an animal model using the Stiff-Man serum.