To differentiate starts from epileptic spasms, we recruited children with brief \"crescendo-decrescendo\" muscle contractions that simultaneously involved the limbs and trunk without electroencephalogram changes, and that fulfilled the following criteria: (1) repeated occurrence (five or more) and (2) manifestation during sleep stage N1-N2. A total of nine children met these criteria. Their clinical information and video-electroencephalogram data were analyzed retrospectively.
The background conditions observed at onset of RSS were perinatal hypoxic-ischemic encephalopathy (n = 4), West syndrome of unknown etiology (n = 1), and traumatic brain injury (n = 1). The age at onset of RSS, the number of starts in a given RSS cluster, the interval between starts, and the duration of surface electromyogram activity were between 3 and 46 months, 5 and 547, <1 and 60 s, and 0.3 and 5.4 s, respectively. None of the median value of these parameters differed between children with and without corticospinal tract injury. During the median follow-up period of 33 months, RSS disappeared spontaneously in five.
This is the largest case series of RSS clarifying their clinicoelectroencephalographic characteristics reported to date. To avoid unnecessary antiepileptic therapies, clinicians should be aware of RSS and distinguish it from other disorders involving involuntary movements or seizures, especially epileptic spasms.
为了区分癫痫性痉挛,我们招募了短暂的“渐增-渐增”肌肉收缩的儿童,这些肌肉收缩同时涉及四肢和躯干,没有脑电图变化,并且满足以下标准:(1)重复发生(五次或更多次)和(2)在睡眠N1-N2阶段表现。共有9名儿童符合这些标准。回顾性分析其临床信息和视频脑电图数据。
RSS发病时观察到的背景条件是围产期缺氧缺血性脑病(n=4),病因不明的韦斯特综合征(n=1),和创伤性脑损伤(n=1)。RSS的发病年龄,给定RSS群集中的开始数,开始之间的间隔,表面肌电图活动的持续时间在3到46个月之间,5和547,<1和60s,0.3和5.4s,分别。这些参数的中值在有和没有皮质脊髓束损伤的儿童之间没有差异。在33个月的中位随访期内,RSS在五次中自发消失。
这是迄今为止报道的最大的RSS系列病例,阐明了其临床脑电图特征。为了避免不必要的抗癫痫治疗,临床医生应该了解RSS,并将其与其他涉及非自主运动或癫痫发作的疾病区分开来,尤其是癫痫性痉挛.