Benign Focal Epileptiform Discharges of Childhood (BEPK) and Lesion of the White Matter of the Brain: The Case from Practice

Background/Purpose: A typical example of BEPK is rolandic epilepsy with centrotemporal spike-sharp waves (electrical 5-point dipole with high amplitude). According to H. Doose, there are two main causes of BEPK: genetic predisposition and organic disorder of the CNS with congenital disorder of brain maturation. The pathology of white matter in the perinatal period is manifested by a delay in the process of myelination with impaired brain maturation. Pathological changes in the white matter of the brain may be a cause of the development of BEPK in the EEG.

Methods: We report on our 6-year-old patient who is examined and treated in our center (clinical, neurological examinations, video EEG, amplitude mapping, frequency mapping). Anamnesis: a long-term tobacco consumption and evidence of drug abuse in the mother's history. Urgent cesarean section was performed. Premature baby was delivered at 28 weeks’ gestation, with birthweight of 960 g. Sepsis. In the further course, bilateral cerebral palsy developed. At the age of 8 months, epilepsy developed with transition to West syndrome. Anticonvulsants (valproic acid) with methylprednisolone pulse therapy. Without seizures. Condition after West syndrome.

Results: Currently significant developmental retardation. Cerebral palsy, bilateral spastic type, grade IV. Without seizures. The slowed EEG showed a transformation from hypsarrhythmia in the context of West syndrome to BEPK with temporal dominance on the right. BEPK does not go away with open eyes indicating an organic white matter lesion.

Conclusion: In the present case, BEPK is a marker of the congenital disorder of brain maturation due to perinatal lesion of the white matter of the brain. BEPK should be considered not always as a marker of epilepsy, but as an expression of congenital disorders in the processes of brain maturation.

Publication History

Article published online:
28 October 2021