CORTICOSTEROID TREATMENT IN LANDAU-KLEFFNER SYNDROME
Abstract number :
2.126
Submission category :
Year :
2002
Submission ID :
1037
Source :
www.aesnet.org
Presentation date :
12/7/2002 12:00:00 AM
Published date :
Dec 1, 2002, 06:00 AM
Authors :
D. Barry Sinclair, Thomas J. Snyder, Marjorie Berg, Donald Gross, S. Nizam Ahmed, Matt Wheatley, Keith Aronyk, Vivek Mehta, John D.S. McKean, William F. Colmers. Comprehensive Epilepsy Program, University of Alberta, Edmonton, Alberta, Canada
RATIONALE: Landau-Kleffner syndrome (LKS) and its variants such as Continuous Spike Wave Discharge of Sleep (CSWS) are progressive epileptic encephalopathies of childhood. They consist of language (LKS) and/or cognitive/behavioural deterioration (CSWS) in a previously well child with continuous epileptic discharge on EEG (with or without clinical seizures). The treatment of this unusual group of patients is controversial. We report our experience in treating patients with LKS and its variants with corticosteroids.
METHODS: Patients were diagnosed with LKS or one of its variants after language regression or cognitive/behavioural deterioration with an epileptic EEG. Patients were then admitted to our Pediatric Epilepsy Monitoring Unit (PEMU) for 24-hour continuous video EEG monitoring, MRI, SPECT scan, speech/language assessments and neuropsychological testing. After confirmation of the diagnosis the patients received Prednisone 1 mg/kg/day for 6 months. The patients were then reassessed at 6 months, 1 year, then yearly. Follow-up was for 1-10 years (mean 4 years).
RESULTS: Eleven patients, 3 girls, 8 boys were studied. Age of onset ranged from 2 to 11 years (mean 7.5 years). Eight patients had LKS, 2 had CSWS and 1 patient had Pervasive Developmental Delay (PDD) with language regression. Most patients had seizures (8/11), however, 3 patients did not have clinical seizures. The EEG was abnormal in 10 patients and included focal epileptic abnormalities in 2, CSWS in 7, 1 patient with both focal epileptic changes and CSWS, and 1 patient (with PDD) was normal. MRI was normal in all patients. SPECT scan was abnormal in 5 patients, normal in 3 and not available in 3. All but one patient had significant improvement in language, cognition and behaviour which continued after the corticosteroids. Side effects were few (4/11) and transient and consisted of weight gain (2), behavioural change (1) and hypertension (1).
CONCLUSIONS: Corticosteroids are a safe and effective treatment for patients with LKS. Most patients have significant improvement in language, cognition and behaviour following treatment. Side effects are few and reversible and benefits long lasting. Corticosteroids should be considered as a treatment option in children with LKS and its variants.