单纯疱疹病毒性脑炎复发伴舞蹈症与抗 N-甲基-D-天冬氨酸受体或多巴胺-2 受体自身抗体有关。
Herpes simplex encephalitis relapse with chorea is associated with autoantibodies to N-Methyl-D-aspartate receptor or dopamine-2 receptor.
机构信息
Neuroimmunology Group, Institute for Neuroscience and Muscle Research, Kids Research Institute at the Children's Hospital at Westmead, University of Sydney, Westmead, Australia; Department of Neurosciences, Royal Children's Hospital, Brisbane, Australia; School of Medicine, University of Queensland, Brisbane, Australia; TY Nelson Department of Neurology and Neurosurgery, Children's Hospital at Westmead, Westmead, Australia.
出版信息
Mov Disord. 2014 Jan;29(1):117-22. doi: 10.1002/mds.25623. Epub 2013 Oct 1.
BACKGROUND
Movement disorder relapses after herpes simplex virus 1 (HSV1) encephalitis have been hypothesized to be secondary to postviral autoimmunity. Recently, a proportion of patients with HSV1 encephalitis (HSE) were shown to produce autoantibodies against N-methyl-D-aspartate receptor (NMDAR).
METHODS
We measured autoantibodies against NMDAR and dopamine-2 receptor (D2R) expressed at the cell surface in the stored acute serum of 9 children with HSE, 3 of whom had a relapsing course with chorea.
RESULTS
The 3 patients with chorea had elevated autoantibodies against NMDAR (n = 1), D2R (n = 1), or both (n = 1), whereas patients without chorea were negative (n = 6). The prospectively identified patient with chorea and NMDAR autoantibodies improved after early treatment with steroids, intravenous immunoglobulin, and cyclophosphamide, with reduction in serum NMDAR antibody titers.
CONCLUSIONS
These autoantibody findings lend support to the autoimmune hypothesis and the early use of immune suppression in post-HSE chorea.
背景
单纯疱疹病毒 1（HSV1）脑炎后运动障碍复发，推测是继发于病毒后自身免疫。最近，一部分 HSV1 脑炎（HSE）患者被发现产生针对 N-甲基-D-天冬氨酸受体（NMDAR）的自身抗体。
方法
我们在储存的 9 例 HSE 儿童的急性血清中测量了针对 NMDAR 和多巴胺-2 受体（D2R）的自身抗体，其中 3 例出现舞蹈症复发。
结果
3 例舞蹈症患者存在 NMDAR（n=1）、D2R（n=1）或两者（n=1）的自身抗体升高，而无舞蹈症的患者为阴性（n=6）。前瞻性识别的舞蹈症和 NMDAR 自身抗体患者在早期接受类固醇、静脉注射免疫球蛋白和环磷酰胺治疗后病情改善，血清 NMDAR 抗体滴度降低。
结论
这些自身抗体发现支持自身免疫假说，并支持早期在 HSE 后舞蹈症中使用免疫抑制治疗。
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