Fine AL, Wirrell EC, Wong-Kisiel LC, Nickels KC.
Acetazolamide for electrical status epilepticus in slow-wave sleep. Epilepsia. 2015
Sep;56(9):e134-8.
Abstract
Electrical status epilepticus in slow-wave sleep (ESES) is
characterized by nearly continuous spike-wave discharges during non-rapid eye
movement (REM) sleep. ESES is present in Landau-Kleffner syndrome (LKS) and
continuous spike and wave in slow-wave sleep (CSWS). Sulthiame has demonstrated
reduction in spike-wave index (SWI) in ESES, but is not available in the United
States. Acetazolamide (AZM) is readily available and has similar pharmacologic
properties. Our aims were to assess the effect of AZM on SWI and clinical
response in children with LKS and CSWS. Children with LKS or CSWS treated with
AZM at our institution were identified retrospectively. Pre- and posttherapy
electroencephalography (EEG) studies were evaluated for SWI. Parental and
teacher report of clinical improvement was recorded. Six children met criteria
for inclusion. Three children (50%) demonstrated complete resolution or SWI
<5% after AZM. All children had improvement in clinical seizures and
subjective improvement in communication skills and school performance. Five of
six children had subjective improvement in hyperactivity and attention. AZM is
a potentially effective therapy for children with LKS and CSWS. This study
lends to the knowledge of potential therapies that can be used for these
disorders, which can be challenging for families and providers.
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