Gurcharran Kevin, Karkare Shefali
Pediatric Neurology, Cohen Children's Medical Center, New Hyde Park, New York.
Pediatric Neurology, Cohen Children's Medical Center, New Hyde Park, New York.
Pediatr Neurol. 2017 Jan;66:104-107. doi: 10.1016/j.pediatrneurol.2016.10.001. Epub 2016 Oct 11.
N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated condition that has a broad spectrum of manifestations, including seizures, coma, psychosis, and focal neurological deficits. Although usually a diffuse process, unihemispheric involvement mimicking early stages of Rasmussen encephalitis can occur. Rasmussen's encephalitis is a unique syndrome characterized by progressive hemiplegia, drug-resistant focal epilepsy, cognitive decline, and hemispheric brain atrophy contralateral to the hemiplegia.
We describe a two-year-old girl with progressive right weakness and epilepsia partialis continua, concerning for early Rasmussen's encephalitis, who tested positive for anti-NMDA receptor antibodies. She experienced complete clinical recovery after immunotherapy. Anti-NMDA receptor antibodies were absent at three weeks and again at one year after the first treatment of intravenous immunoglobulin.
There are few reports of Rasmussen-like encephalitis in individuals with anti-NMDA receptor antibody positivity. Thus the clinical significance of this association is yet to be determined. In addition, several other antibodies have been documented in individuals with Rasmussen encephalitis. The lack of a consistently reported antibody in Rasmussen encephalitis patients and the temporary nature of the anti-NMDA receptor antibody in our patient raise the following question: Is the presence of anti-NMDA receptor antibodies the cause of the symptoms or secondary to the pathogenic process?
N-甲基-D-天冬氨酸（NMDA）受体脑炎是一种免疫介导的疾病，具有广泛的临床表现，包括癫痫发作、昏迷、精神病和局灶性神经功能缺损。虽然通常是弥漫性病变，但也可能出现单侧半球受累，类似于拉斯穆森脑炎的早期阶段。拉斯穆森脑炎是一种独特的综合征，其特征为进行性偏瘫、药物难治性局灶性癫痫、认知功能减退以及与偏瘫对侧的半球脑萎缩。
我们报告一名两岁女童，出现进行性右侧肢体无力和持续性部分性癫痫发作，怀疑为早期拉斯穆森脑炎，其抗NMDA受体抗体检测呈阳性。免疫治疗后她实现了完全临床康复。在首次静脉注射免疫球蛋白治疗后三周及一年时，抗NMDA受体抗体均未检测到。
抗NMDA受体抗体阳性个体中类似拉斯穆森脑炎的病例报告较少。因此，这种关联的临床意义尚待确定。此外，在拉斯穆森脑炎患者中还发现了其他几种抗体。拉斯穆森脑炎患者缺乏一致报道的某种抗体，且我们患者体内抗NMDA受体抗体具有暂时性，这引发了以下问题：抗NMDA受体抗体的存在是症状的原因还是致病过程的继发表现？
