7. Conclusions

MMPSI is an independent epileptic syndrome with special clinicalneurophysiological characteristics, distinct from other forms of epilepsy. Diagnosis can be established if there are different types of focal seizures, involving multiple extended EEG and electro-clinical ictal EEG patterns with involvement of several independent areas in both hemispheres. All the patients need complex investigations including dynamic video-EEG monitoring, neuroimaging, and genetic tests (whole-exome sequencing is more preferable).

MMPSI should also be differentiated from the syndrome described by Ohtahara – "severe epilepsy with multiple independent spike foci" (SE-MISF). In the literature, this form is also known as Markand-Blume-Ohtahara syndrome [37]. Unlike MMPSI, this form manifests with predominantly pseudogeneralized seizures: bilateral axial tonic spasms, atypical absences, and myoclonic but focal seizures could also observed. But SE-MISF and MMPSI could have evolutional changes into each other.

Therefore, malignant migrating partial seizures of infancy is the third type of infantile epileptic encephalopathy, along with early encephalopathies with suppression-burst pattern (Aicardi and Ohtahara syndromes) and West syndrome, when the cerebral cortex is more prone to generate epileptic excitation migrating from one area of the cortex to another, without clear interregional organization. This condition is caused by age-dependent features of infant brain with cortex hyperexcitability at a certain stage of evolution [3, 38].

The definition of this syndrome has not been defined in the international classification of epilepsies and epileptic syndromes. The term "malignant migrating partial seizures of infancy" characterizes this form of epilepsy rather as syndrome, so it is proposed to discuss the following title "malignant epilepsy of infancy with migrating multifocal seizures" that may more fully capture the essence of the disease. Taking into account contributions of scientists that first described this form of epilepsy (Coppola) and gave the most detailed description of the clinical and neurophysiological criteria (Dulac), the following definition is proposed: Coppola-Dulac syndrome [39].
