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Department of Pediatrics, University of Alabama at Birmingham School of Medicine, Birmingham, AL (Drs. Snead and Benton)
Centre de Neurochimie du INSERM et CNRS U44, Strasbourg, France (Dr. Snead)
Comprehensive Epilepsy Center for Children, The Alabama Children's Hospital, Birmingham, AL (Dr. Snead and Ms. Hosey)
Wadsworth Center for Laboratories and Research, State of New York Department of Health, Albany, NY (Drs. Swann, Spink, and Martin, and Mr. Rej).
Fifteen children with infantile spasms and a hypsarrhythmic EEG defined by EEG-videotelemetry monitoring received a regimen of high-dose (150 IU/m2/d) ACTH for their seizures. We carried out an endocrinologic evaluation before and after initiation of the ACTH and conducted a time course study of plasma ACTH and cortisol levels after ACTH dosing. Spasms were controlled and the EEG normalized in 14 of the 15 children. Prior to starting ACTH therapy all the patients had normal prolactin, insulin, cortisol, and ACTH levels in plasma and normal thyroid function. Although the pattern of rise of ACTH levels in plasma after ACTH dosing was similar in all the children, there was great individual variation in the absolute concentrations. However, both the pattern of rise and absolute level of cortisol in plasma after ACTH was highly predictable in all patients. Plasma cortisol rose rapidly within 1 hour of ACTH administration and continued a slower rise for 12 to 24 hours after the ACTH dose. High-dose ACTH therapy seems quite effective in infantile spasms, perhaps because of a sustained high level of plasma cortisol. This sustained plateau of cortisol may be more effective in controlling infantile spasms than the pulse effect expected with oral steroids or lower doses of ACTH.
Address correspondence and reprint requests to Dr. Snead, 4650 Sunset Blvd., PO Box 54700, Los Angeles, CA 90054-0700.
Supported in part by grant No. F06 TWO 1277 from the Fogarty International Foundation (to O.C.S.).
Received December 9, 1988. Accepted for publication in final form February 14, 1989.
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