Abstracts

RATIONALE: Continuous Spike Wave in Sleep (CSWS) is a rare condition associated with mental status changes and Landau-Kleffner Syndrome. Treatment methodology remains anecdotal. We report two cases where EEG improved on prednisone and valproic acid, but with ACTH high dose therapy, EEG normalized and clinical improvements were observed.
METHODS: Two patients received high dose (150 units/meter ^2) ACTH on a daily schedule, slowly tapered every two weeks. Serum electrolytes, glucose, and CBC were monitored bi-weekly, with blood pressure monitored weekly. Patient 1: Five year old male presented with language regression, myoclonic and generalized convulsions increasing in frequency from 36 months of age. Prior development was normal. Patient failed prednisone, valproic acid, lamotrigine, and vigabatrin. Patient was mute and had two grand mal seizures per week. Initial EEG showed continuous spike and slow wave activity at 2-3 Hz with undifferentiated sleep states. Patient 2: Eight year old male regressed at 4 years of age with atypical, prolonged absence seizures, mental regression of speech and cognitive slowing. Patient was in special education with a 4-5 year old functioning level. Patient had previously failed valproic acid, ethoxysuccimide and prednisone. Initial EEG showed frequent semicontinuous sleep activated CSWS, with 2-3 Hz delta or spike and wave activity.
RESULTS: ACTH therapy significantly improved EEG background in both patients, with both experiencing minor hypokalemia and weight gain of 5-12 pounds. Patient 1 remained mute, with his EEG still showing intermittent generalized polyspikes. Patient 2 had dramatic improvement in responsiveness, awareness of the environment, and complete normalization of EEG.
CONCLUSIONS: These two cases show an alternative treatment for refractory CSWS patients with documented EEG normalization with high dose ACTH therapy.