The Correlation of Clinical Findings of Neuronal Migration Disorders with Brain Magnetic Resonance Imaging Characteristics / 대한소아신경학회지

ABSTRACT

PURPOSE: Beginning in the eighth week of fetal life the neuroblasts migrate from the midline to the periphery to form the gray matter of the cerebral cortex. Abnormalities of cell migration are characterized by ectopic location of neurons in the cerebral cortex. This broad group of anomalies include lissencephaly, schizencephaly, cortical dysplasia, gray matter heterotopia, and unilateral hemimegalencephaly. The purpose of this study was to correlate clinical data with anatomic data, which was evaluated by brain magnetic resonance imaging characteristics that are most useful in predicting clinical METHODS: The clinical records, EEG, and MRI findings of 20 patients with neuronal migration disorders were retrospectively reviewed. RESULTS: The 20 patients with neuronal migration disorders consisted of 11 with lissencephaly, 7 with cortical dysplasia, 2 with heterotopia, and 2 with schizencephaly. Clinically, seizure was the most common symptom in 85%, next developmental delay in 50%, and then delayed speech in 25%, motor deficit 15% in order. The main associated brain anomalies included absence of septum pellucidum in 20%, periventricular leukomalacia in 15%, and corpus callosal agenesis in 15% of Patients. Bilateral involvement of lesion in MRI was 60%, comparing to unilateral lesion in 40% of the patients. The most common involved lobes was frontoparietal region. An abnormality of EEG examination was showed in 11 cases of patients(68.7%). Patients with diffuse, bilateral lesion in MRI findings of neuronal migration disorders had significantly developmental delay than those with unilateral lesion(p=0.0007). Patients with unilateral lesion had significantly motor deficit than those with bilateral lesion(p=0.04). CONCLUSION: Seizures were the most common symptoms among neurological manifestations of neuronal migration disorders. Statistically significant correlations of delayed developement with bilateral lesion and motor deficit with unilateral lesion were found.