High Dose ACTH in Lennox-Gastaut Syndrome Varients.
Abstract number :
1.175
Submission category :
Year :
2001
Submission ID :
609
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
K. Schaefer, R.N., Pediatric Neurology, SC., Lake Bluff, IL; M.G. Chez, M.D., Pediatric Neurology, SC., Lake Bluff, IL; R.J. Tremb, B.A., Pediatric Neurology, SC., Lake Bluff, IL
RATIONALE: Lennox-Gastaut has a grim prognosis for both seizure control and cognitive outcome.
METHODS: Ten consecutive patients (6 females, 4 males, 1-8 years of age) presented with intractable myoclonic and mixed seizures, and/or atypical absence slow spike and wave seizures. Patients were treated with high dose ACTH therapy after failing at least two standard anticonvulsants, one of which was valproic acid. Patients had EEG activity with 1-3 Hz slowing, intermixed with polyspikes or prolonged electrographic 2-3 Hz paradoxical delta activity, consistent with Lennox-Gastaut phenotype. Patients received high dose (150 units/meter ^2) ACTH on a daily schedule, slowly tapered every two weeks. In six of 10 patients, we lowered the frequency of injections to 1-2 per week for maintenance.
RESULTS: Seven patients became seizure free, with only four having seizure recurrence after being off ACTH for 8-12 months. Two patients remain medication free. The remaining uncontrolled patients still had dramatic seizure reduction, with improvement in EEG background to remove the Lennox-Gastaut EEG pattern. Four patients remain on pulse dose ACTH maintenance every 3-4 days. Two patients tapered off ACTH successfully.
CONCLUSIONS: Older children with Lennox-Gastaut Syndrome or intractable atypical absence seizures can respond with EEG and clinical improvement in seizure control and cognitive performance to high dose ACTH therapy.