Sakuma Hiroshi
Developmental Neuroimmunology Project, Homeostasis in Brain Development, Tokyo Metropolitan Institute of Medical Science.
Brain Nerve. 2018 Apr;70(4):363-369. doi: 10.11477/mf.1416201009.
Autoimmune basal ganglia disorders (ABGDs) are presumed autoimmune encephalitides characterized by movement disorders and basal ganglia lesions on neuroimaging. The most common type of autoimmune encephalitis manifesting as movement disorders is anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. Anti-phospholipid antibody syndrome and neuropsychiatric lupus may present with chorea or other involuntary movements. In childhood, Sydenham's chorea is an important differential diagnosis. Although autoantibodies directed against the surface antigens on basal ganglia neurons are assumed to cause ABGDs, few autoantibodies have been demonstrated to be relevant to certain clinical syndromes except for anti-NMDA receptor antibodies. However, recent studies have identified autoantibodies to the dopamine D2 receptor and collapsin response mediator proteins in patients with ABGDs. It remains to be elucidated, however, whether these autoantibodies to basal ganglia antigens play pathogenic roles in ABGDs.
自身免疫性基底节疾病（ABGDs）被认为是自身免疫性脑炎，其特征为运动障碍以及神经影像学上的基底节病变。表现为运动障碍的最常见自身免疫性脑炎类型是抗N-甲基-D-天冬氨酸（NMDA）受体脑炎。抗磷脂抗体综合征和神经精神性狼疮可能表现为舞蹈症或其他不自主运动。在儿童期， Sydenham舞蹈症是一个重要的鉴别诊断。尽管针对基底节神经元表面抗原的自身抗体被认为可导致ABGDs，但除抗NMDA受体抗体外，很少有自身抗体被证明与某些临床综合征相关。然而，最近的研究已在ABGDs患者中鉴定出针对多巴胺D2受体和塌陷反应介导蛋白的自身抗体。然而，这些针对基底节抗原的自身抗体是否在ABGDs中起致病作用仍有待阐明。
