[SP3-3A-4] Surgical Treatment and Long-term Seizure and Developmental Outcome of Infantile Epileptic Encephalopathies Associated with Cortical Dysplasia
Infantile epileptic encephalopathies (IEE) associated with cortical dysplasia (IEE+CD) are severe disorders. Long-term seizure and developmental outcome one to 15 years after surgery in patients with IEE+CD were compared to those in patients with IEE without CD (IEE-CD) treated non-surgically for one to 33 years. Resective or disconnective surgery was applied to IEE+CD: 22 cases of Ohtahara syndrome [OS: 20 with hemimegalencephaly (HME), 2 with multilobar dysplasia (MCD)], early myoclonic encephalopathy 1 [EME: MCD 1], West syndrome 18 [WS: HME 4, MCD 8, focal cortical dysplasia (FCD) 6], Aicardi syndrome 1 [AS: MCD 1], and 17 nonspecific IEE [NIEE: MCD 9, FCD 8]. IEE-CD included 15 cases of OS, 9 EME, 47 WS and 4 AS. Surgical procedures included hemisherotomy 25, multilobar disconnection 20, and focal resection 14. Surgery was done at 58 days and under 12 months in 52, and one and 3 years in 7. Non-surgical treatment included antiepileptic drugs, ACTH therapy, IVIG, and ketogenic diet. At the last evaluation, seizure outcome for ISS+CD/SS-CD were: seizure free (SzF) 18 cases/6 cases, weekly seizures (WSz) 1/1, daily seizures (DSz) 3/8 in OS; DSz 1/9 in EME; SzF 13/16, monthly seizures (MSz) 0/3, WSz 2/4, DSz 3/24 in WS; DSz 1/4 in AS; SzF 16, DSz 1 in NIEE．For IEE+CD/IEE-CD, IQ/DQ>70 were 12 cases/2 cases and <20 10/64, bed-ridden 7/50, and dead during follow-up period 1/8. Seizure and developmental outcome were far better in IEE+CD than those in IEE-CD. For IEE+CD, early surgery is encouraging for good outcome.