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抗 N-甲基-D-天冬氨酸受体脑炎相关孤立性皮质下病灶:病例报告。

Solitary juxtacortical lesion associated with anti-N-methyl-D-aspartate receptor encephalitis: a case report.

机构信息

Department of Hematology, Zhongshan Hospital, Fudan University, Shanghai, China.

Department of Neurology, Huashan Hospital, Fudan University, No. 12 Mid Urumqi Road, Jin'an District, Shanghai, 200040, China.

出版信息

BMC Neurol. 2020 Nov 20;20(1):421. doi: 10.1186/s12883-020-01997-6.

Abstract

BACKGROUND

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a severe autoimmune encephalitis mediated by anti-NMDA receptor antibodies. Brain MRI manifestations vary and are non-specific. If there are any lesions, they tend to be diffusely or multifocally distributed. Solitary lesion is relatively rare.

CASE PRESENTATION

We report a 16-year-old girl who initially presented with focal seizures but developed severe psychiatric and extrapyramidal symptoms later on. Brain MRI revealed a solitary juxtacortical demyelinating lesion in the left frontal lobe. No enhancement was noted. Electroencephalogram captured epileptiform discharges in the same region. NMDAR IgGs were tested positive in the serum and cerebrospinal fluid. Corticosteroid and intravenous IgG were administered and the patient completely recovered. Brain MRI revealed a fainter lesion in the left frontal lobe.

CONCLUSION

In very rare instances, anti-NMDA receptor encephalitis can present with a solitary brain lesion. A full panel of antibodies for autoimmune encephalitis is the key leading to the diagnosis.

摘要

背景

抗 N-甲基-D-天冬氨酸(NMDA)受体脑炎是一种由抗 NMDA 受体抗体介导的严重自身免疫性脑炎。脑 MRI 表现多样且非特异性。如果有任何病变,它们往往呈弥漫性或多灶性分布。单发病变相对少见。

病例介绍

我们报告了一例 16 岁女孩,最初表现为局灶性发作,但后来出现严重的精神和锥体外系症状。脑 MRI 显示左额叶皮质下脱髓鞘病变。未见强化。脑电图在同一区域捕捉到癫痫样放电。血清和脑脊液中 NMDA 受体 IgGs 检测阳性。给予皮质类固醇和静脉注射免疫球蛋白后,患者完全康复。脑 MRI 显示左额叶病变较前稍淡。

结论

在非常罕见的情况下,抗 NMDA 受体脑炎可表现为单发脑病变。自身免疫性脑炎的全套抗体是诊断的关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6607/7678186/474dc5ea122e/12883_2020_1997_Fig1_HTML.jpg

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