Medical Faculty, University of Ljubljana, Slovenia.
Eur J Paediatr Neurol. 2012 Jan;16(1):64-73. doi: 10.1016/j.ejpn.2011.10.006. Epub 2011 Nov 23.
During early development severe epilepsies may appear, some with well established occurrence. Benign non-epileptic and epileptic paroxysmal syndromes with excellent prognosis occur in the same period. There are no exact data on their occurrence.
We have reviewed medical histories of children with benign non-epileptic or benign epileptic events: benign myoclonus of early infancy, benign neonatal sleep myoclonus, benign sleep myoclonus in infancy, benign partial epilepsy in infancy (BPEI) and benign infantile familial convulsions (BIFC) were established. The occurrence, clinical characteristics and prognosis of these syndromes were evaluated.
Inclusion criteria were met in 31 children. Research included retrospective analysis of clinical characteristics, laboratory values, neuroimaging and neurophysiological assessments, followed by evaluation of psychosocial development with the use of the Strengths and Difficulties Questionnaire (SDQ), fulfilled by parents.
In our group the incidence of benign non-epileptic convulsions was 6.69 per 10 000 live births and the incidence of benign epileptic convulsions was 1.35 per 10 000. Male/female ratio in the group of children with non-epileptic events was 2.1:1. Among non-epileptic group 5 out of 23 children and among epileptic group 3 out of 8 children had minimal, mild or moderate abnormalities at neurological assessment at the time of the first clinical examination. Nonspecific changes in laboratory values were seen in 6 out of 23 in the non-epileptic and in 1 out of 8 children in the epileptic group. Neurophysiological assessments showed subtle changes in 4/23 in the non-epileptic and 6/8 in the epileptic group. Neuroimaging was not optimal in 5/23 with non-epileptic and 3/8 with epileptic events. Analysis of SDQ did not show significant deviations in psyhosocial development. Statistically significant deviation was observed only in relations with peers (p = 0.009).
Benign neonatal and infantile convulsions are more frequent than severe epilepsies of the same age period. Results show higher proportion of males with benign non-epileptic conditions. No deviations in further development was found. Laboratory values, neuroimaging and neurophysiological assessments were normal or nonspecifically changed.
在早期发育过程中,可能会出现严重的癫痫,其中一些具有明确的发病机制。同时,在同一时期还存在预后良好的良性非癫痫性和癫痫性阵发性综合征。目前,这些疾病的发生频率尚无确切数据。
我们回顾了患有良性非癫痫性或良性癫痫性事件的儿童的病历,包括良性婴儿早期肌阵挛、良性新生儿睡眠肌阵挛、良性婴儿期睡眠肌阵挛、良性婴儿期局灶性癫痫(BPEI)和良性婴儿家族性惊厥(BIFC)。评估这些综合征的发生、临床特征和预后。
31 名儿童符合纳入标准。研究包括对临床特征、实验室值、神经影像学和神经生理学评估进行回顾性分析,随后使用父母填写的长处和困难问卷(SDQ)评估社会心理发展情况。
在我们的研究组中,良性非癫痫性惊厥的发病率为每 10000 例活产儿 6.69 例,良性癫痫性惊厥的发病率为每 10000 例 1.35 例。非癫痫组 23 例患儿中,男/女比例为 2.1:1;癫痫组 8 例患儿中,男/女比例为 3:1。首次临床检查时,非癫痫组 5 例患儿和癫痫组 3 例患儿的神经评估有轻微、轻度或中度异常。非癫痫组 23 例患儿中有 6 例和癫痫组 8 例患儿中有 1 例的实验室值有非特异性改变。非癫痫组 23 例患儿中有 4 例和癫痫组 8 例患儿中有 6 例的神经生理学检查有细微改变。非癫痫组 23 例患儿中有 5 例和癫痫组 8 例患儿中有 3 例的神经影像学检查不理想。SDQ 分析未显示社会心理发育有显著偏差。仅在与同龄人交往方面观察到统计学显著偏差(p=0.009)。
良性新生儿和婴儿惊厥比同期严重癫痫更为常见。结果显示,良性非癫痫性疾病中男性比例较高。进一步的发育没有发现偏差。实验室值、神经影像学和神经生理学检查结果正常或无特异性改变。