"Obtundation Status (Dravet)" Caused by Complex Partial Status Epilepticus in a Patient with Severe Myoclonic Epilepsy in Infancy

Summary: Purpose : We report a 1-year 7-month-old boy with severe myoclonic epilepsy in infancy (SME) who exhibited complex partial status epilepticus (CPSE), which was confirmed by ictal video-EEG analysis. This boy first had a hemi-convulsion in a hot bath at age 3 months. Thereafter, he exhibited both partial and generalized seizures that were extremely intractable. At age 9 months, he had a prolonged episode of impaired consciousness that fit the "obtundation status in SME" defined by Dravet et al.
Methods and Results : Ictal EEG revealed persisting irregular spike-and-wave complexes over the left hemisphere, predominantly in the occipitotemporal area, and confirmed CPSE. The EEG abnormalities with decreased level of the consciousness continued -6 h after onset of the symptoms even with AED administration.
Conclusions : Because SME features both generalized and focal seizures, both types of nonconvulsive status may be seen in SME. Although Dravet et al. already reported long-lasting atypical absences in patients with SME as "obtundation status," we demonstrated CPSE in an infant with SME who exhibited a prolonged stuporous state.