The place of neuronal migration abnormalities in child neurology.
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.
Aicardi
J
J
Hôpital des Enfants Malades, Paris, France.
D002465:Cell Movement; D002648:Child; D006801:Humans; D009422:Nervous System Diseases / Q000175:diagnosis / Q000209:etiology / Q000473:pathology*; D009474:Neurons