Neuropediatrics 2006; 37 - THP164
DOI: 10.1055/s-2006-945987

NEURONAL MIGRATION DISORDERS – CORRELATION BETWEEN CLINICAL AND NEUROIMAGING FEATURES

R Teleanu 1, D Plesca 1, F Buruiana 1, AM Davitoiu 1, I Stancea 1, L Cretu 1, D Dragomir 1
  • 1ILAE, Bucharest, Romania

Objectives: Neuronal migration disorders are a group of malformations of the brain which primarily affect development of the cerebral cortex and it consists of agyriapachygyria, polymicrogyria, schizencephaly, heterotopia and focal cortical dysplasia. This study aimed to investigate the clinical characteristics and diagnostic methods of neuronal migration disorders. Methods: We analyze the clinical and neuroimaging features of 33 patients diagnosed with neuronal migration disorders. Patients were evaluated from the clinical, electroencephalographic (EEG) and neuroradiological point of view. Their ages at the time of the first presentation ranged from 7 weeks to 14 years.

Results: 19 patients were diagnosed as lissencephaly, 12 patients as polymicrogyria – schizencephaly complex and 2 patients with focal cortical dysplasia. 24 patients (73%) experienced epileptic seizures with onset during first year of life. 16 patients (67%) had epileptic seizures resistant to conventional treatment. Clinically, 26 patients (78.7%) had various types of motor deficits, 17 patients (51.5%) had microcephaly, and 8 children (24%) had facial dysmorphism. Mental retardation was observed in 28 cases (85%) and was severe in 25 cases. Conclusion: The results of this study suggest that neuronal migration disorders are characterized by epilepsy, mental retardation and motor deficits. This malformations should be investigated in the etiology of early-onset childhood epilepsy and it should be considered in children with developmental delay with or without microcephaly and facial dysmorphism.