Pediatrics & Therapeutics
Open Access
ISSN: 2161-0665
+44 1478 350008
ISSN: 2161-0665
+44 1478 350008
P. Nagarjuna and H. Swamy Rajesh
Posters: J Pediatr Therapeut
Lissencephaly, which literally means smooth brain, is a rare brain formation disorder caused by defective neuronal migration during the early weeks of gestation, resulting in a lack of development of sulci and gyri. The final proper layer formation relies on an intricate balance between events of the cell cycle, proliferation, neuronal path findings and migration. The lissencephaly syndromes in humans involve abnormal cortical lamination and are categorized as neuronal migration defects. We report two cases of 10 year old, male identical twins, born to apparently healthy consanguineous parents with no significant antenatal, perinatal or immediate postnatal complications who were brought with complaints of delayed developmental milestones, mental retardation and seizures. Seizures were generalized tonic clonic type and started at 4 years of age in both the twins. Examination revealed microcephaly, mental retardation, hypertonia and spasticity with gait abnormality. In both the twins CT BRAIN revealed features of agyria ? pachygyria complex (lissencephaly) with dysmyelination changes. MRI BRAIN revealed : Classic type I lissencephaly with band heterotopia. The clinical features & neuroimaging studies are consistent with the findings of Type 1 Lissencephaly in both the identical twins. Key Words: Identical twins, seizures, lissencephaly