Outcome of synthetic adrenocorticotropin hormone treatment in children with infantile spasm

Main Article Content

I Gusti Ngurah Made Suwarba

Abstract

Background Infantile spasms (IS) is an age-spedfic epilepsy syndrome characterized by flexor, extensor, and mixed flexor-extensor spasms which often occur in clusters during the first 2 years of life. IS is often difficult to manage 'With the usual anti-epilepsy drugs (AEDs). Therapy with adrenocorticotropin honnone (ACTH) has been used since 1958. In Indonesia, ACTH usage is still rare.

Objective This study aims to examine the effectiveness of ACTH as an anti-epileptic drug in managing IS.

Methods This was descriptive retrospective cohort study. Subjects were IS patients who visited the neurology outpatient clinic in Sanglah Hospital, Bali, from January 2007 until June 2010. Each subject received AED(s) plus either ACTH or methylprednisolone for 4􀁆6 weeks.

Results There were 19 IS patients over the four year duration of this study. They were mostly boys (11), aged 2 weeks to 17 months, with a mean age at treatment of 9 months. Eighteen patients received poly therapy, while one patient received only phenobarbital as monotherapy. Most patients who received ACTH (13/16) had a seizure-free period, while the 3 that did not receive ACTH continued having seizures. Patients who received ACTH

showed a good response (seizure-free) after 5-13 days therapy and their EEG pattern showed disappearance of burst suppression Mthin 1-2 weeks. ACTH side effects included weight gain and cushingoid appearance. One patient died from pneumonia.

Conclusions Diagnosis of IS should be considered in patients pre-senting Mth spasms at less than 6 months old. IS treatment should begin as soon as possible. IS patients responded well to a short course of ACTH therapy. 

Article Details

How to Cite
1.
Suwarba IGN. Outcome of synthetic adrenocorticotropin hormone treatment in children with infantile spasm. PI [Internet]. 30Apr.2011 [cited 22Apr.2021];51(2):94-00. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/894
Section
Articles
Received 2016-10-13
Accepted 2016-10-13
Published 2011-04-30

References

1. Mackay MT, Weiss SK, Adams-Webber TS, Ashwal SD, Stephens D, Ballaban􀁤Gill K, et al. Practice parameter: medical treatment of infantile spasms: Report of the American Academy of Neurology and the Child Neurology Society.N eurology. 2004;62: 1668-81.
2. Shields WD. Infantile spasms: an overview.J Pediatr Neurol. 2004;2:1-3.
3. RHkonen R. Epidemological data of West syndrome in Finland. Brain Dev. 2001;23:539-41.
4. Fois A. Infantile spasms: review of the literature and personal experience. Italian J Pediatr. 2010;36: 1-10.
5. Tsao CY. Current trend in the treatment of infantile spasms. J Neuropsych Dis Treat. 2009;5:289-99.
6. Karvelas G, LortieA, Scantlebury MH, Duy PT, Cossette P, Cannant L. A retrospective study on aetiology based outcome of infantile spasms. Seizure. 2009;18:197-201.
7. Wong M, Trevathan E. Infantile spasms: article review. Pediatr Neurol. 2001;24:89-98.
8. Shanna NL, Viswanathan V. Outcome in West syndrome. Indian Pediatr. 2008;45:559-63.
9. Setyo H, Irawan M. Manifestasi klinik dan tatalaksana spasme infantil di Departemen Hmu Kesehatan Anak F KUI􀁤RSCM Jakarta. Sari Pediatri. 2007;8:21-6.
10. Riikonen R. A long-term follow-up study of 214 children with the syndrome of infantile spasm. Neuropediatr. 1982;13:14-23.
11. Fusco I, Vigevano F. Ictal clinical electroencephalographic finding of spasms in West syndrome. Epilepsia . 1993;34:671-8.
12. Arzimanoglou A, Guerrini R, AicardiJ. Infantile spasms and related syndrome. In: Arzimanoglou A, Guerrini A, Aicardi J, editors. Aicardi's epilepsy in children. 3rd ed. Philadelphia: Lippincott Williams & Wilkins; 2004. p.14-37.
13. Gauser TA. Infantile spasms (West syndrome). [cited 2010 June 15]. Available from: http:Uwww.eMedicineNeurology file:IJFIS 121Sl.htrn.
14. Ahmed R. Comparative study of corticotrophin vs vigabatrin therapy in infantile spasms. Pak J Med Sci. 2007;23:141A.
15. Azam M, Bhatti N, Krishin J. Use of ACTH and prednisolone in infantile spasms: experience from a developing country. Seiwre. 2005;14:552-6.
16. Lux AI, Edwards Sw, Hancock E, Johnson AI, Kennedy CR, Newton RW. The United Kingdom infantile spasms study comparing vigabatrin 'With prednisolone or tetracosactide at 14 days: a multicenter, randomized controlled trial. Lancet. 2004;364: 1773-8.
17. Mitchell WG, Shah NS. Vigabatrin for infantile spasms. PediatrN eurol. 2002;27:161A.
18. Holden KR, Clarke SL, Griesemer DA. Long􀁤term outcome of conventional therapy for infantile spasms. Seizure. 1997;6:201-5.
19. Gupta R, Appleton R. Corticosteroid in the management of the paediatric epilepsies. Arch Dis Child. 2005 ;90:379-84.
20. Ismail S. Outcome of patients 'With infantile spasms. Paediatr Indones. 2000;40:30A.
21. Golomb MR, Garg BP, Williams LS. Outcome of children with infantile spasms after perinatal stroke. Pediatr Neurol. 2006;34:291-5.
22. Guggenheim lvtA, Frost JD, Hrachovy RA. Time interval from a brain insult to the onset of infantile spasms. Pediatr Neurol. 2008;38:34-7.
23. Riikonen R. Steroid or vigabatrin in the treatment infantile spasm. Pediatr Neurol. 2000;23:403-8.
24. Commission on Pediatric Epilepsy of The International League Against Epilepsy. Workshop on infantile spasms. Epilepsia.1992;33,195-9.
25. Korff CM, N ordli DR. Epilepsy syndrome in infancy. Pediatr Neurol. 2006;34,253-63.
26. Shang NX, Zou LP, Zhao JB, Zhang F, Li H. Association between prenatal stress and infantile spasms: a case control study in China. Pediatr Neurol. 2010;42:181-6.
27. Primec ZR, Krivec JL, Krivec U, Neubauer D. Head growth in infants 'With infantile spasms may be temporarily reduced. Pediatr Neurol. 2006;35,197-203.
28. Ozawa H, Kawada Y, Noma S, Sugai K. Oral high-dose phenobarbital therapy for early infantile epileptic encephalopathy. Pediatr Neurol. 2002;26:222A.