Malignant migratory partial seizures of infancy: description of clinical observations

Epileptic encephalopathies of infancy are difficult-to-treat destructive cases of epilepsy in infants and young children, which include the two clinical observations of Coppola – Dulac syndrome (malignant migrating partial attacks of infancy) given in this article. Epileptic encephalopathies are a genetically heterogeneous group of disorders that are characterized by the presence of a convulsive syndrome and are accompanied by cognitive and behavioral disorders. As a result of intensive development of methods of molecular genetic research, ideas about the causes of epilepsy in children began to change, this fact led to the fact that the international League against epilepsy replaced the term “idiopathic epilepsy” with “genetic epilepsy”. To date, about 60 phenotypic variants of early epileptic encephalopathies have been registered in children, including those associated with mutations of genes that regulate the functions of voltage-dependent calcium and sodium channels. In our work, we consider clinical observations of genetic epileptic encephalopathies caused by mutations in the CACNA1A and SCN8A genes, respectively, which are channelopathy. Malignant migrating partial attacks of infancy (infant epilepsy with migrating focal attacks, Coppola – Dulac syndrome) are manifested at an early age, from the first days to 6 months of life, and are characterized by typical multifocal attacks with a tendency to change hemispheres and involve opposite limbs. G. Coppola based on the analysis of their own observations, described in 1995 a new age – dependent epileptic syndrome, introducing a definition for it – “migrating partial attacks of infancy”. Currently, it is recommended to use the term “infant epilepsy with migrating focal seizures”. We present our own clinical and neurophysiological observations of this rare syndrome.