Abstract

LISSENCEPHALY (SMOOTH BRAIN): A COMPREHENSIVE REVIEW ASSOCIATED WITH CYTOMEGALOVIRUS (CMV)

*Mrs. M. Anitha, K. Elavarasan, T. Madhan Kumar, R. Nigila Sri, S. Ramadevi

Abstract

A rare congenital cortical abnormality called lissencephaly is characterized by decreased or missing cerebral gyri as a result of poor neuronal migration in the early stages of pregnancy. It is linked to severe neurodevelopmental damage and manifests as a spectrum between Agyria and Pachygyria. Although a significant percentage of cases are caused by genetic abnormalities involving genes including LIS1, DCX, TUBA1A, and RELN, non-genetic causes—particularly congenital infections—play an important and frequently overlooked role. Congenital cytomegalovirus (CMV) infection is the most important viral etiology because of its great preference for brain progenitor cells and the germinal matrix. Particularly in the first and early second trimesters, maternal CMV infection can interfere with neuronal migration and proliferation, resulting in lissencephaly and related symptoms like microcephaly, seizures, sensorineural hearing loss, and global developmental delay. Prenatal and postnatal neuroimaging, laboratory confirmation of CMV infection, and genetic testing to distinguish infectious from inherited causes are used in the diagnosis process. Early detection of CMV-associated disease allows for prompt antiviral medication and specialized supportive care, which may improve certain outcomes even though lissencephaly is still incurable. To lower the burden of disease, preventive approaches such as genetic counselling, prenatal screening, and maternal hygiene practices are crucial.

Keywords: Lissencephaly, Neuronal migration, Congenital cytomegalovirus [CMV], Cortical malformation, Antiviral therapy.