A large body of experience has been compiled in different countries, documenting the efficacy of adenocorticotropic hormone (ACTH) for infantile spasms. This is important, because it may serve as a key for understanding this disorder, as well as for designing better medicines. However, significant discrepancies exist among studies originating in different countries regarding the relative efficacy of small or large ACTH doses.
These differences may be caused by a number of factors, including potential genetic or environmental-related differences in the biology of the disorder or associated genetic components that determine responsiveness to ACTH. In addition, striking differences in the preparations used around the world may be responsible. These include bio-availability and extent of blood brain barrier penetration, efficacy in activating the efficacy-mediating ‘ACTH receptors’, the presence in certain preparations of competing analogs, and others. These issues should not detract from the overall agreement that ACTH might be the most useful medication currently available to treat WS.
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- Double-blind study of ACTH vs prednisone therapy in infantile spasms.J Pediatr. 1983; 103: 641-645
- Infantile spasms.Pediatr Clin North Am. 1989; 36: 311-329
- A pilot study of topiramate in the treatment of infantile spasms.Epilepsia. 1998; 39: 1324-1328
- ACTH therapy for infantile spasms: a combination therapy with high-dose pyridoxal phosphate and low-dose ACTH.Epilepsia. 1998; 39: 42-45
- Vitamin B6 and valproic acid in treatment of infantile spasms.Pediatr Neurol. 1991; 7: 91-96
- High dose ACTH versus prednisone for massive infantile spasms: a prospective randomized blinded study.Pediatrics. 1996; 97: 375-379
- Treatment of infantile spasms.Pediatr Neurol. 1990; 3: 147-150
- Liposteroid (dexamethasone palmitate) therapy for West syndrome: a comparative study with ACTH therapy.Pediatr Neurol. 1998; 18: 415-419
- A comparative study of high-dose and low-dose ACTH therapy for West syndrome.Brain Dev. 1999; 21: 461-467
- Treatment of infantile spasms with long-term low dose ACTH.No To Hattatsu (Tokyo). 1992; 24 (in Japanese): 469-474
- A study on ACTH dosage for treatment of West syndrome.No To Hattatsu (Tokyo). 1993; 25 (in Japanese): 215-220
- ACTH acts directly on amygdala neurons to down-regulate corticotropin releasing hormone gene expression.Ann Neurol. 2001; 49: 304-313
- West syndrome: individualized ACTH therapy.Brain Dev. 1996; 18: 456-460
- Pathophysiology of massive infantile spasms (MIS): perspective on the role of the brain adrenal axis.Ann Neurol. 1993; 33: 231-237
Accepted: August 21, 2001
Received in revised form: July 12, 2001
Received: June 11, 2001
© 2001 Published by Elsevier Inc.