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一名年轻肯尼亚女性患预后评分差的致命性自身免疫性抗N-甲基-D-天冬氨酸受体脑炎

Fatal Autoimmune Anti-NMDA-Receptor Encephalitis with Poor Prognostication Score in a Young Kenyan Female.

作者信息

Rakiro Joe, Sokhi Dilraj

机构信息

Department of Medicine, Aga Khan University Medical College of East Africa, Nairobi, Kenya.

出版信息

Int Med Case Rep J. 2021 May 24;14:343-347. doi: 10.2147/IMCRJ.S311071. eCollection 2021.

Abstract

Auto-immune N-methyl-D-aspartate receptor encephalitis (NMDARE) is a relatively recently described cause of acute encephalopathy with very few reports from sub-Saharan Africa (SSA). We report a case of NMDARE in a young Kenyan female who was transferred to our facility with headaches, insomnia, behaviour changes and latterly pathognomonic orofacial dyskinesias. We comprehensively ruled out infectious and other inflammatory/auto-immune causes. She was diagnosed with NMDARE by positive antibody testing in serum and cerebrospinal fluid and changes on brain magnetic resonance imaging. She was immunosuppressed with high-dose steroids, intravenous immunoglobulins, plasma exchange and rituximab, and showed signs of neurological improvement clinically and radiologically. Unfortunately, she succumbed to septic shock from prolonged intensive care. This is the first report of NMDARE in an indigenous patient from the eastern SSA. The majority (>80%) of patients are either left with mild disability or make a full recovery after NMDARE, but some factors - which comprise the NMDARE One-Year Functional Status (NEOS) prognostication score - can adversely affect outcome, as was the case in our patient.

摘要

自身免疫性 N-甲基-D-天冬氨酸受体脑炎(NMDARE)是一种相对较新发现的急性脑病病因,撒哈拉以南非洲(SSA)地区关于该病的报道极少。我们报告了一例年轻肯尼亚女性的 NMDARE 病例,该患者因头痛、失眠、行为改变以及后来出现的特征性口面部运动障碍被转诊至我们的机构。我们全面排除了感染性及其他炎症/自身免疫性病因。通过血清和脑脊液中抗体检测呈阳性以及脑磁共振成像的改变,她被诊断为 NMDARE。她接受了大剂量类固醇、静脉注射免疫球蛋白、血浆置换和利妥昔单抗的免疫抑制治疗,临床和影像学上均显示出神经功能改善的迹象。不幸的是,她因长期重症监护导致感染性休克而死亡。这是东非 SSA 地区本土患者中首例 NMDARE 报告。大多数(>80%)患者在患 NMDARE 后要么遗留轻度残疾,要么完全康复,但一些因素——构成 NMDARE 一年功能状态(NEOS)预后评分——可能对结局产生不利影响,我们的患者就是这种情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f26b/8164334/b08c58054176/IMCRJ-14-343-g0001.jpg

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