Bartolomei F, Gavaret M, Dhiver C, Gastaut J A, Gambarelli D, Figarell-Branger D, Gastaut J L
Centre Saint-Paul for Epilepsy, Department of Neurophysiology and Neuropsychology, Université de la Méditerranée, Marseille, France.
Arch Neurol. 1999 Jan;56(1):111-4. doi: 10.1001/archneur.56.1.111.
The characteristic clinical feature of epilepsia partialis continua (EPC) is chronic focal myoclonus, usually involving the distal part of one extremity. A variety of pathogenetic factors have been implicated in EPC. In children, the most common cause is Rasmussen encephalitis; in adults, it is vascular disease or tumor involving the sensorimotor cortex. Epileptic seizures are a relatively common manifestation of central nervous system involvement in patients infected with human immunodeficiency virus (HIV), but, to our knowledge, isolated, chronic EPC has not been previously reported.
To describe a case of typical EPC in a patient infected with HIV.
Case report from an epilepsy center.
A 58-year-old man infected with HIV had continuous myoclonus that involved the right arm and was associated with intermittent motor seizures. The electroencephalographic findings were normal at the onset of the symptoms, but left central theta rhythm appeared later. Serial magnetic resonance imaging scans obtained over a 3-month period showed a progressively increasing left rolandic T2-weighted hypersignal. Histologic study of a stereotactic biopsy specimen demonstrated inflammation characterized by perivascular mononuclear cell infiltration. The only detectable cause was HIV infection. Immunocytochemical tests ruled out JC virus. Neuropsychological testing showed no evidence of cognitive impairment. An electroencephalographic-electromyographic "back-averaging" study showed a reproducible transient left biphasic complex preceding the bursts by about 30 milliseconds on the C3 and F3 electrodes, thus demonstrating that the myoclonus was of cortical origin. High-dose corticosteroid (prednisone, 100 mg/d) and anti-HIV- 1 therapy led to marked radiological and clinical improvement. Infection with HIV enhances the risk of seizures, but, to our knowledge, this is the first reported case of "inflammatory" EPC.
The present case suggests that the possibility of central nervous system involvement by HIV-1 should be taken into account in the diagnostic workup of patients with EPC. This case also indicates that treatment can be effective.
持续性部分性癫痫(EPC)的特征性临床特点是慢性局灶性肌阵挛,通常累及一个肢体的远端。多种致病因素与EPC有关。在儿童中,最常见的病因是拉斯穆森脑炎;在成人中,是累及感觉运动皮层的血管疾病或肿瘤。癫痫发作是人类免疫缺陷病毒(HIV)感染患者中枢神经系统受累的相对常见表现,但据我们所知,此前尚未报道过孤立的慢性EPC。
描述1例HIV感染患者的典型EPC病例。
来自癫痫中心的病例报告。
一名58岁的HIV感染男性,出现累及右臂的持续性肌阵挛,并伴有间歇性运动性发作。症状发作时脑电图检查结果正常,但后来出现左侧中央θ节律。在3个月内进行的系列磁共振成像扫描显示左侧中央前回T2加权高信号逐渐增加。立体定向活检标本的组织学研究显示以血管周围单核细胞浸润为特征的炎症。唯一可检测到的病因是HIV感染。免疫细胞化学检测排除了JC病毒。神经心理学测试未显示认知障碍的证据。脑电图 - 肌电图“反向平均”研究显示,在C3和F3电极上,爆发前约30毫秒有可重复的短暂左侧双相复合波,从而证明肌阵挛起源于皮层。大剂量皮质类固醇(泼尼松,100mg/d)和抗HIV-1治疗导致明显的影像学和临床改善。HIV感染会增加癫痫发作的风险,但据我们所知,这是首例报告的“炎症性”EPC病例。
本病例提示,在EPC患者的诊断检查中应考虑HIV-1累及中枢神经系统的可能性。该病例还表明治疗可能有效。