Koul Roshan L, Alfuitasi Amna M, Sankhla Dilip K, Javad Hashim, William Ranjan R
Department of Child Health, Sultan Qaboos University Hospital, PO Box 38, BW-1, Muscat 123, Sultanate of Oman. Fax. +968 24413128. E-mail:
Neurosciences (Riyadh). 2009 Apr;14(2):158-62.
To record the pattern of different neuronal migrational disorders (NMD) and their associated neurological conditions.
The data were collected at the Child Neurology Services of Sultan Qaboos University Hospital, Oman, from January 1993 to September 2006 from all children with psychomotor delay and epilepsy, who underwent brain imaging (mostly MRI). The MR imaging was used for the diagnosis of a neuronal migration anomaly.
There were 86 cases of NMD. Corpus callosum agenesis and lissencephaly/pachygyria formed the major group. There were 48 cases of corpus callosum agenesis, and 16 cases of lissencephaly/pachygyria. Other disorders were 10 cases of heterotopias, 5 schizencephaly, 3 holoprosencephaly, 2 polymicrogyria, and one each of hemimegalencephaly, and hydranencephaly. Developmental delay was the most common associated finding noted in 80 (93%) cases. Sixty-seven (77.9%) cases had motor deficit. Forty out of 86 (46.5%) cases had epilepsy. Partial/partial complex seizures were the most common at 13 out of 40 (32.5%). Syndromic seizures were seen in 11 out of 40 (27.5%) cases. The seizures were controlled in only 3/40 (7.5%) cases.
The NMD constitute a significant number of child neurology patients with psychomotor delay and intractable epilepsy. Exogenic and genetic factors affecting the early embryonic and fetal development from sixth to twenty-sixth weeks of gestation result in NMD. Recent genetic studies are defining the underlying mechanism and these studies will help in early diagnosis and possible prevention of NMD.
记录不同神经元迁移障碍（NMD）的模式及其相关的神经系统疾病。
数据收集于阿曼苏丹卡布斯大学医院儿童神经科，时间跨度为1993年1月至2006年9月，收集对象为所有患有精神运动发育迟缓及癫痫且接受了脑部成像检查（大多为MRI）的儿童。磁共振成像用于诊断神经元迁移异常。
共有86例NMD病例。胼胝体发育不全和无脑回/巨脑回形成了主要群体。胼胝体发育不全有48例，无脑回/巨脑回有16例。其他疾病包括10例异位症、5例脑裂畸形、3例前脑无裂畸形、2例多小脑回畸形，以及各1例半侧巨脑畸形和积水性无脑畸形。发育迟缓是最常见的相关表现，80例（93%）出现该症状。67例（77.9%）有运动功能障碍。86例中有40例（46.5%）患有癫痫。部分性/部分复杂性发作最为常见，40例中有13例（32.5%）为此类型。40例中有11例（27.5%）出现综合征性发作。仅3/40例（7.5%）的癫痫得到控制。
NMD构成了患有精神运动发育迟缓和难治性癫痫的大量儿童神经科患者。影响妊娠第6至26周早期胚胎和胎儿发育的外源性和遗传因素导致了NMD。近期的基因研究正在明确其潜在机制，这些研究将有助于NMD的早期诊断和可能的预防。
