THE PLACE OF NEURONAL MIGRATION ABNORMALITIES IN CHILD NEUROLOGY
THE PLACE OF NEURONAL MIGRATION ABNORMALITIES IN CHILD NEUROLOGY
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DOI：
10.1017/s0317167100041159
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发表时间：
1994-08-01
影响因子：
3
通讯作者：
AICARDI, J
中科院分区：
文献类型：
--
作者：
AICARDI, J
随着现代影像学技术的发展，神经元迁移障碍已成为儿童癫痫、智力低下和慢性神经功能障碍的主要原因。本文报告69例，其中弥漫性迁移异常46例，局部发育不良23例。弥漫性迁移障碍患者表现为智力迟钝、大运动障碍和严重的癫痫发作障碍，而局灶性异常患者以癫痫为主诉。磁共振成像，虽然通常诊断迁移障碍往往不允许病理类型的定义。一些脑电图模式，如高振幅快节奏或θ - δ模式是高度暗示性的。大多数异常迁移病例是偶发的，可能是获得性的。有些是由于染色体异常，特别是染色体17p，其中一个无脑畸形基因已被定位。家族性病例既有隐性遗传，也可能有显性遗传。由于移动异常引起的癫痫通常是难以治疗的。切除发育不良的皮质可能对局部疾病有效，而胼胝体切开术已被建议用于弥漫性异常。
With the development of modern imaging techniques, disturbances of neuronal migration appear to be a major cause of epilepsy, mental retardation and chronic neurological disability in childhood. Sixty-nine cases are presented, including 46 of diffuse migration abnormalities and 23 of localized dysplasia. Patients with diffuse migration disorders presented with mental retardation, gross motor impairment and severe seizure disorders whereas in those with focal anomalies, epilepsy was the chief complaint. Magnetic resonance imaging, although usually diagnostic of migration disorders often does not allow definition of the pathologic type. Some EEG patterns, such as high amplitude fast rhythms or the theta-delta pattern are highly suggestive. Most cases of abnormal migration are sporadic and probably acquired. Some are due to chromosomal anomalies, especially of chromosome 17p where a gene for lissencephaly has been mapped. Familial cases occur with both recessive and possibly dominant inheritance. Epilepsy due to migration abnormalities is often intractable. Resection of dysplastic cortex may be effective for localized disease and callosotomy has been proposed for diffuse anomalies.