LISSENCEPHALY: INTERDISCIPLINARY TREATMENT

Abstract

Miller–Dieker syndrome, also known as lissencephaly, is a genetic disorder characterized by shallow cerebral sulci and thickened, fewer, or absent gyri. Few cells effectively reach the cerebral cortex, and most neurons have their migration interrupted, remaining in an ectopic position. This results in a lack of mechanical stimulation for the formation of gyri, leading to severe neurological impairment, including profound intellectual disability, spasticity or hypotonia, and multifocal epilepsy or epilepsy with secondary generalization, which is associated with seizures that are extremely refractory to treatment. From this perspective, the syndrome requires interdisciplinary treatment aimed at maximizing childhood development and improving the quality of life of patients and their families. Therefore, this article aims to compile findings on lissencephaly and discuss its treatments, with an emphasis on epilepsy, cognition, and motor development, elucidating the methods that have been previously investigated.