McKeon Andrew, Lennon Vanda A, Pittock Sean J
Continuum (Minneap Minn). 2010 Apr;16(2 Dementia):80-101. doi: 10.1212/01.CON.0000368213.63964.34.
The diagnosis of an autoimmune dementia requires the detection of objective improvements in cognitive decline (usually subacute in onset with a fluctuating course) after a course of immunotherapy. Serum and CSF antibody markers of autoimmunity (particularly those with neural antigen specificity) as well as other CSF markers of inflammation increase the suspicion for an autoimmune cause. The detection of neural autoantibodies should raise concern for a paraneoplastic etiology and may inform a targeted oncologic evaluation (eg, NMDA receptor antibodies are associated with teratoma). MRI, EEG, functional imaging, and neuropsychological evaluations provide objective evidence of neurologic dysfunction by which the success of immunotherapy may be measured. Most treatment information emanates from retrospective case series and expert opinion. Nonetheless, early intervention allows reversal of deficits in many patients. Chronic treatment is often required to maintain remission.
自身免疫性痴呆的诊断需要在免疫治疗疗程后检测到认知功能下降（通常起病亚急性，病程波动）有客观改善。自身免疫的血清和脑脊液抗体标志物（特别是那些具有神经抗原特异性的标志物）以及其他脑脊液炎症标志物会增加对自身免疫病因的怀疑。神经自身抗体的检测应引起对副肿瘤病因的关注，并可为有针对性的肿瘤学评估提供依据（例如，NMDA受体抗体与畸胎瘤相关）。MRI、脑电图、功能成像和神经心理学评估提供神经功能障碍的客观证据，据此可衡量免疫治疗的效果。大多数治疗信息来自回顾性病例系列和专家意见。尽管如此，早期干预可使许多患者的缺陷得到逆转。通常需要长期治疗以维持缓解状态。
