Multiple brain cavernoma presenting as myoclonic astatic seizures in a pediatric patient: Case report

Abstract

Myoclonic astatic epilepsy is a unique epilepsy syndrome constituting approximately 1–2% of childhood-onset epilepsies with 94% of the cases occurs within first 5 years of life. Seizures were reported as myoclonic jerking movement continued by a brief atonia. EEG may be initially normal and may show polyspike and wave complexes with progression of the disease. Myoclonic astatic epilepsy was known to be genetically involved, we would like to report a case with similar seizure pattern in a patient with structural abnormality. A 3-year-old male patient presented to the emergency department with progressing disequilibrium and headache since 1 month before admission. Headache, frequent staring and jerking followed by brief falling were also seen. This was the first onset seizure. Patient had a complete immunization schedule. History of previous illness was denied. Neurological examination showed myoclonic jerks followed by atonia, hence no weakness of the extremities. Routine laboratory findings was unremarkable and electroencephalogram study showed multifocal epileptiform discharges in both frontal region, left parietal and right temporal region. Head MRI with contrast showed multiple cavernoma in both cerebral hemisphere and bilateral lateral periventricle with the diameter of 0.6–2.66 cm. Patient was then discharged on valproic acid and had controlled seizure until now. Myoclonic astatic seizures is a rare condition with potential genetic cause. This case shows that structural abnormalities might be a cause of similar seizure pattern. Valproic acid is shown to be effective in controlling seizure of this patient.