Abstract
Atypical benign partial epilepsy (ABPE) of childhood is a special type of epileptic
syndrome characterized by the combination of epileptic negative myoclonus, atypical
absence seizures, focal motor seizures, and a continuous spike–wave during slow sleep
(CSWS). Although the seizures are resistant to antiseizure drugs (ASDs) effective
for focal epilepsy, they are markedly responsive to ethosuximide (ESM). An incorrect
ASD choice may aggravate the seizures and CSWS, resulting in a pseudo-catastrophic
state or nonconvulsive status epilepticus. In this study, we retrospectively investigated
the effectiveness of steroid and ketogenic diet (KD) therapies for children with ABPE
resistant to ASDs, including ESM. Adrenocorticotropic hormone (ACTH) therapy (n = 4), KD therapy (n = 4), and an oral steroid (n = 2) were tried for eight patients (two patients tried two therapies). An excellent
response (seizure-free period > 1 year) was achieved by four out of four patients
undergoing ACTH therapy, two out of four undergoing KD therapy, and one out of two
receiving oral steroid therapy. In conclusion, steroid and KD therapies are reasonable
treatment options early in the course of treatment of children with pharmacoresistant
ABPE to prevent a decline in cognitive function due to the persistence of epileptic
encephalopathy. The majority of patients in our case series had an excellent response,
though further studies are warranted to confirm their efficacy and safety.
Keywords
atypical benign partial epilepsy - CSWS - ACTH therapy - ketogenic diet - ethosuximide
