Landau-Kleffner syndrome and its variants such as continuous Spike-Wave Discharge during Sleep (CSWS) are progressive epileptic
encephalopathies of childhood. The treatment of this unusual group of patients is controversial. We describe our experience in treating patients with
Landau-Kleffner syndrome and CSWS with
corticosteroids. The patients received
Prednisone 1 mg/kg/day for 6 months, 1 year, then yearly. Follow-up was for 1-10 years (mean 4 years). Ten patients, 3 females, 7 males were studied. Age of onset ranged from 2 to 11 years (mean 7.5 years). Eight patients manifested
Landau-Kleffner syndrome, and two had CSWS. Most patients had
seizures (8/10); however, two patients did not have clinical
seizures. MRI was normal in all patients. SPECT scan was abnormal in four patients, normal in three, and not available in three. All but one patient manifested significant improvement in language, cognition, and behaviour, which continued after the
corticosteroid trial. Side effects were few (4/10) and transient and consisted of
weight gain (2), behavioral change (1), and
hypertension (1).
Corticosteroids are a safe and effective treatment for patients with
Landau-Kleffner syndrome and CSWS. Most patients had improvement in language, cognition, and behaviour
after treatment. Side effects are few and reversible, and benefits appear long lasting.
Corticosteroids should be considered as a treatment option in children with
Landau-Kleffner syndrome and CSWS.