Child Neurology Department, Pediatric Faculty of Russian State Medical University, Moscow, Russia.
Acta Neurol Scand. 2012 May;125(5):345-52. doi: 10.1111/j.1600-0404.2011.01575.x. Epub 2011 Jul 19.
Epilepsia partialis continua (EPC) is characterized by localized continuous jerks, from time to time with spreading Jacksonian seizures and, more rarely, secondarily generalized tonic-clonic seizures. EPC has numerous possible etiologies. In this paper we describe EPC in the tick-borne Russian spring-summer encephalitis (TBRSSE) and compare it with Rasmussen syndrome.
We included patients with EPC in TBRSSE (between 2003 and 2010). The diagnosis was verified by immunology (antibodies against TBRSSE virus). The patients were followed 1-7 (mean 3.4) years.
We studied 10 patients (eight males, age 10-21 years) with MRI and video-EEG. Nine developed EPC after acute TBRSSE (meningoencephalitic form), and one had a tick bite without clinical symptoms of encephalitis, but with subsequent EPC. All patients came from Ural and Siberia. The onset was at age 4-14 (mean 8.6 years). The interval from onset of TBRSSE or the tick bite to seizure onset was 1 day-4 years. We identified three phases of clinical course EPC in TBRSSE: (i) acute (meningoencephalitic/encephalitic); (ii) development of EPC; and (iii) chronic EPC. The effect of antiepileptic drugs differed according to seizure types.
EPC caused by TBRSSE is relatively frequent in the Eastern parts of the Russian Federation but not west of the Ural. Unlike Rasmussen encephalitis, EPC with TBRSSE does not progress even in the long term. It appears as disabling but not fatal condition with a time course where three phases can be distinguished.
部分连续性癫痫发作(EPC)的特征是局部持续抽搐,不时伴有扩散性杰克逊癫痫发作,更罕见的是继发全身性强直-阵挛发作。EPC 有许多可能的病因。本文描述了蜱传俄罗斯春夏脑炎(TBRSSE)中的 EPC,并将其与 Rasmussen 综合征进行了比较。
我们纳入了 TBRSSE 中的 EPC 患者(2003 年至 2010 年)。诊断通过免疫学(针对 TBRSSE 病毒的抗体)证实。患者随访 1-7 年(平均 3.4 年)。
我们研究了 10 例 MRI 和视频脑电图患者(8 名男性,年龄 10-21 岁)。9 例患者在急性 TBRSSE 后出现 EPC(脑膜脑炎型),1 例患者有蜱咬史而无脑炎临床症状,但随后出现 EPC。所有患者均来自乌拉尔和西伯利亚。发病年龄为 4-14 岁(平均 8.6 岁)。从 TBRSSE 或蜱咬到发作的间隔为 1 天至 4 年。我们发现 TBRSSE 中 EPC 的临床病程有三个阶段:(i)急性(脑膜脑炎/脑炎);(ii)EPC 发展;和(iii)慢性 EPC。抗癫痫药物的效果根据发作类型而有所不同。
TBRSSE 引起的 EPC 在俄罗斯东部地区较为常见,但在乌拉尔以西则不常见。与 Rasmussen 脑炎不同,TBRSSE 引起的 EPC 即使在长期内也不会进展。它表现为一种致残但非致命的疾病,其病程可分为三个阶段。