Clinical Features of Cerebral Cortex Malformations in Children: A Study in Upper Egypt

Abstract

Malformation of Cortical Development (MCD) corresponds to a broad spectrum of cerebral lesions resulting from cortical development abnormalities during embryogenesis. MCD are increasingly recognized as an important cause of epilepsy, especially refractory ones. Aims: Evaluation of pattern and clinical spectrum of MCD. Patients and methods: The data of children presented to the Pediatric Department, or Pediatric Neurology Clinic, Sohag University Hospital whom Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) brain showing MCD were collected retrospectively regarding their history, examinations, and developmental parameters, Electroencephalography (EEG), Intelligence Quotient (IQ). Results: during the study periods we reviewed the data of 50 Child with MCD, the mean age of the study group was 3.7 years with age range from 4 days to 15 years. The majority of the patients (50%) belonged to the age group 1-5 years old. Convulsions were the main complaint in the majority of children 21 (42%) out of 50 child, followed by global developmental delay in 20 out of 50 child. Seizures generally were present in (82%). Concerning type of seizures, multiple seizure types were found in 16 (39%) out of 41 child, partial seizures found in 13 (31.7%), generalized tonic in 6 (14.6%), infantile spasm in 4 (9.8%), and generalized tonic-clonic in 2 (4.8%). Types of malformation of cortical development in the studied children as defined by neuro-imaging, (42%) had lissencephaly, (16%) had bilateral Schizencephaly, (12%) had unilateral schizencephaly, (12%) had polymicrogyria, (10%) had focal cortical dysplasia, (6%) had periventricular heterotopia, and one child with subcortical laminar heterotopia. Conclusion: The common cortical malformations found in Sohag University Hospital were lissencephaly, bilateral Schizencephaly, unilateral schizencephaly. Also the most common clinical features of MCDs in our community are refractory seizures and/or global developmental delay, and the most valuable tool in diagnosis is brain MRI. Recommendations: Informing pediatricians to raise their clinical suspicion to MCDs in children presented by frequent, intractable, early onset, and partial or multiple types’ seizures and/or global developmental delay, and that MRI brain must be done then.