Department of Neurology, Bethesda Children's Hospital, Budapest, Hungary.
Epilepsia. 2012 Mar;53(3):565-70. doi: 10.1111/j.1528-1167.2012.03404.x. Epub 2012 Feb 14.
Psychogenic nonepileptic seizure (PNES) is an important differential diagnostic problem in patients with or without epilepsy. There are many studies that have analyzed PNES in adults; currently, however, there is no systematic assessment of purely childhood PNES semiology. Our study based on a large pediatric video-electroencephalography (EEG) monitoring (VEM) cohort, provides a detailed analysis of childhood PNES and assesses the usability of the current classification system described in adults.
Medical and video-EEG records of 568 consecutive children (younger than 18 years) who underwent video-EEG monitoring (VEM) at our hospital were reviewed. Aura, type of movement, anatomic distribution, synchrony, symmetry, eye movement, responsiveness, vocalization, hyperventilation, vegetative and emotional signs, presence of eyewitness, and duration of the event were recorded among children with the diagnosis of PNES. We also compared our data with those of earlier adult studies.
Seventy-five archived PNES of 27 children (21 girls; age 8-18 years) were reanalyzed. Nine children (33%) had the diagnosis of epilepsy currently or in the past. Mean age at the time of PNES onset was 11.6 (standard deviation 3.2) years. Mean duration of PNES was longer (269 s) compared to seizures of the epileptic group (83 s; p = 0.002). Eyewitnesses (mostly parents) were present in 89% of cases. Eighty percent of PNES had an abrupt start, with 68% also ending abruptly. In only 15% of events were the patients eyes closed at the beginning of the attack. Patients were unresponsive in 34%. The most frequent motor sign was tremor (25%) with the upper, rather than lower limbs more frequently involved. Pelvic thrusting was seen in only two attacks. Emotional-mostly negative-signs were observed during 32 PNES (43%). Based on Seneviratne et al.'s classification, 18 events (24%) were classified as rhythmic motor PNES, only half the frequency of that previously described in adults. No hypermotor PNES was found. The frequency of complex motor PNES (13%) and mixed PNES (4%) showed similar frequency in children as in adults. Dialeptic PNES was found more frequently among younger children. All PNES belonged to the same semiologic type in 23 patients (85%).
Because homogeneity of PNES within a patient was high in the pediatric population, we found it useful to classify PNES into different semiologic categories. Dialeptic PNES seems to be more frequent among younger children. Tremor is the most frequent motor sign and usually accompanied by preserved responsiveness in childhood. Negative emotion is commonly seen in pediatric PNES, but pelvic thrusting is a rare phenomenon. We, therefore, suggest a modification of the present classification system in which PNES with motor activity is divided into minor and major motor PNES, and the latter group is subdivided into synchron rhythmic motor and asynchron motor PNES. We believe that our study, a detailed analysis on the semiology and classification of purely childhood PNES might assist the early and precise diagnosis of nonepileptic paroxysmal events.
在有或没有癫痫的患者中，心因性非癫痫性发作（PNES）是一个重要的鉴别诊断问题。有许多研究分析了成人的 PNES；然而，目前尚无对纯粹儿童 PNES 症状学的系统评估。我们的研究基于一个大型儿科视频-脑电图（EEG）监测（VEM）队列，对儿童 PNES 进行了详细分析，并评估了目前在成人中描述的分类系统的可用性。
回顾了在我院接受视频-脑电图监测（VEM）的 568 例连续儿童（年龄小于 18 岁）的医疗和视频-EEG 记录。对诊断为 PNES 的儿童记录了先兆、运动类型、解剖分布、同步性、对称性、眼球运动、反应性、发声、过度通气、植物性和情绪体征、目击者存在以及事件持续时间。我们还将我们的数据与早期成人研究进行了比较。
重新分析了 27 名儿童（21 名女孩；年龄 8-18 岁）的 75 个存档的 PNES。目前或过去患有癫痫的儿童有 9 名（33%）。PNES 发作时的平均年龄为 11.6 岁（标准差 3.2）。PNES 的平均持续时间较长（269 秒），而癫痫组的发作持续时间为 83 秒（p=0.002）。目击者（多为父母）在 89%的病例中存在。80%的 PNES 突然发作，68%也突然结束。在仅有 15%的事件中，患者在发作开始时眼睛是闭着的。34%的患者无反应。最常见的运动征象是震颤（25%），以上肢而非下肢更为常见。仅在两次发作中观察到骨盆推挤。32 次 PNES（43%）观察到情绪-主要是负面-迹象。根据 Seneviratne 等人的分类，18 次事件（24%）被归类为节律性运动性 PNES，频率仅为以前在成人中描述的一半。未发现过度运动性 PNES。复杂运动性 PNES（13%）和混合性 PNES（4%）在儿童中的频率与成人相似。在较小的儿童中发现了更多的双相性 PNES。在 23 名患者（85%）中，所有 PNES 属于相同的症状类型。
由于儿科人群中患者内 PNES 的同质性较高，我们发现将 PNES 分为不同的症状学类别很有用。在较小的儿童中，双相性 PNES 似乎更为常见。震颤是最常见的运动征象，通常伴有儿童期的反应性保留。在儿科 PNES 中经常观察到负面情绪，但骨盆推挤是一种罕见现象。因此，我们建议对目前的分类系统进行修改，将有运动活动的 PNES 分为轻微和主要运动性 PNES，后者再分为同步节律性运动和不同步运动性 PNES。我们相信，我们的研究，对纯粹儿童 PNES 的症状学和分类的详细分析，可能有助于非癫痫性阵发性事件的早期和准确诊断。
