Elavarasi Arunmozhimaran, Shukla Garima, Vibha Deepti, Suri Vaishali, Bal Chandra Sekhar
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Department of Medicine, Queen's University, Kingston, ON, Canada.
Ann Indian Acad Neurol. 2022 May-Jun;25(3):495-498. doi: 10.4103/aian.aian_982_21. Epub 2022 Mar 21.
Chronic encephalitis manifesting as an epilepsy syndrome most commonly presents as Rasmussen's syndrome, usually characterized by epilepsia partialis continua, hemiparesis, and progressive cortical deficits such as aphasia, hemianopia, and cognitive decline. It is characterized by progressive hemispheric cortical atrophy on imaging and is usually seen in childhood. Adult-onset of the syndrome is rare, and only a few cases have been reported with bilateral symptoms. We present a patient with pseudobulbar affect and frontal lobe dysfunction who developed multifocal myoclonic jerks, right hemibody focal motor seizures, and right hemiparesis with bilateral cerebellar signs. Magnetic resonance imaging showed progressive hemispheric atrophy and bilateral features in Positron emission tomography-computed tomography (PET CT). Brain biopsy revealed chronic T-cell infiltrate. We discuss this case as the patient had several features that were atypical for Rasmussen's encephalitis (or syndrome).
表现为癫痫综合征的慢性脑炎最常见的是拉斯穆森综合征，通常表现为持续性部分性癫痫、偏瘫以及进行性皮质功能缺损，如失语、偏盲和认知衰退。其影像学特征为进行性半球皮质萎缩，多见于儿童期。该综合征成人发病罕见，仅有少数双侧症状的病例报道。我们报告一例伴有假性球麻痹和额叶功能障碍的患者，该患者出现多灶性肌阵挛性抽搐、右侧半身局灶性运动性癫痫发作以及伴有双侧小脑体征的右侧偏瘫。磁共振成像显示进行性半球萎缩，正电子发射断层扫描-计算机断层扫描（PET CT）有双侧表现。脑活检显示慢性T细胞浸润。我们讨论此病例，因为该患者有几个特征不符合拉斯穆森脑炎（或综合征）的典型表现。
