Research Article
Year 2021,
, 149 - 155, 11.03.2021
Abstract
Amaç: ACTH tedavisi alan West sendromlu hastalarımızın ileri dönemdeki nörogelişimsel prognozu ve nöbet prognozunun saptanması ve bunlara katkı sunan faktörlerin belirlenmesidir.
Gereç ve Yöntem: Kliniğimizde 3-24.aylarında West sendromu tanısı alarak ACTH tedavisi uygulanan 34 hastanın demografik faktörleri, nöbet şekilleri, öncesindeki nöbet öyküleri, ACTH kullanımı ve yanıt süreleri, etyolojik faktörlerini belirledik. Bunların nörolojik gelişim ve nöbet prognozuna etkisini araştırdık.
Bulgular: Hastaların tanı öncesinde nöbeti olmasının ilerideki nöbet prognozu ile ilişkisini anlamlı bulduk. Ayrıca semptomatik etyolojili hastalarda ACTH yanıtını daha geç; nörogelişim ve nöbet prognozunu ise daha kötü saptadık. Tüm hastaların %76’ sında global developmental delay, % 62’sinde antiepileptik ilaclara rağmen devam eden nöbetler belirledik.
Sonuç: West sendromunda semptomatik etyolojik faktörler nörogelişimsel süreci ve ileriki dönemdeki nöbet prognozunu olumsuz etkilemektedir.
References
- 1. Hrachovy RA, Frost JD Jr. Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome). J Clin Neurophysiol 2003;20(06):408–425 2. Swaiman KF, Ashwal S, Ferriero DM, Schor NF, Finkel RS, Gropman LA, Pearl PL, Shevell MI. Swaiman’s pediatric neurology 6th ed. 2017 Elsevier 539-542 3. Kaushik JS, Patra B, Sharma S, Yadav D, Aneja S. Clinical spectrum and treatment outcome of West Syndrome in children from Northern India. Seizure. 2013;22:617–21. 4. Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, et al. Infantile spasms (West syndrome): Update and resources for pediatricians and providers to share with parents. BMC Pediatr. 2012;12:108. 5. Sehgal R, Gulati S, Sapra S, Tripathi M, Kabra M, Pandey RM. Neurodevelopmental and epilepsy outcome in children aged one to five years with infantile spasms—A North Indian cohort. Epilepsy Res. 2014;108:526–34. 6. Riikonen RS. Favourable prognostic factors with infantile spasms. Eur J Paediatr Neurol 2010;14(01):13–18 7. Anlar B ,Yalaz K (1996) Denver II gelişimsel tarama testi Türk çocuklarına uyarlanması ve standardizasyonu el kitabı, Ankara Meteksan Matbası 8. Arzimanoglou A, O’Hare A, Johnston MV, Ouvrier R . Aicardi’s Disease of nervous system in childhood 4th ed. 2018 Mac Keith Press.877-878. 9. Lagae L, Verhelst H, Ceulemans B, De Meirleir L, Nassogne MC, De Borchgrave V, et al. Treatment and long term outcome in West syndrome: The clinical reality. A multicentre follow up study. Seizure. 2010;19:159–64. 10. Yılmaz S, Tekgul H, Serdaroglu G, Akcay A, Gokben S. Evaluation of ten prognostic factors affecting the outcome of West syndrome. Acta Neurol Belg. 2016 Dec;116(4):519-527. 11. Partikian A, Mitchell WG (2010) Neurodevelopmental and epilepsy outcomes in a North American cohort of patients with Infantile spasms. J Child Neurol 25:423–428 12. Araujo AC, Fontenelle LM, Pires L (2011) West syndrome: early remission is not assurance of normal final outcome. Child Care Health Dev 37:261–265 13. Shields WD. Infantile spasms: Little seizures, BIG consequences. Epilepsy Curr. 2006;6:63–9. 14. Riikonen R, Infantile Spasms: Outcome in Clinical Studies . 2020 Feb 4. pii: S0887-8994(20)30041-2 15. Knupp K, Coryel J, Nickels K, et al. Response to treatment in a prospective national infantile spasms cohort. Ann Neurol. 2016;79:475e484. 16. Lux A, Edwards S, Hancock E, et al. The United Kingdom infantile spasm study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomized controlled trial. Lancet. 2004;364:1773e1778. 17. Widjaja E, Go C, McCoy B, Snead OC. Neurodevelopmental outcome of infantile spasms: A systematic review and meta-analysis. Epilepsy Res. 2015 Jan;109:155-62. 18. Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ, et al. Infantile spasms: a U.S. consensus report. . Epilepsia. 2010 Oct;51(10):2175-89. 19. Scheffer I, Berkovic S, Capovilla G, et al. ILAE classification of epilepsies: po- sition paper of the ILEA commission for classification and terminology. Epilepsia. 2017;58:512e521. 20. Riikonen R: Epidemiological data of West syndrome in Finland. Brain Dev 2001, 23:539–541. 21. Trevathan E, Murphy CC, Yeargin-Allsopp M: The descriptive epidemiology of infantile spasms among Atlanta children. Epilepsia 1999, 40:748–751. 22. Webb DW, Fryer AE, Osborne JP: Morbidity associated with tuberous sclerosis: a population study. Dev Med Child Neurol 1996, 38:146–155. 23. Riikonen R (1995) Decreasing perinatal mortality: unchanged infantile spasm morbidity. Dev Med Child Neurol 37:232–238 24. Ibrahim S, Gulab S, Ishaque S, Salem T (2010) Clinical profile and treatment of infantile spasms using vigabatrin and ACTH a developing country perspective. BMC Pediatrics 10:1 25. Mohamed BP, Scott RC, Desai N, Gutta P, Patil S (2011) Seizure outcome in infantile spasms—a retrospective study. Epilepsia 52:746–752 26. Cohen-Sadan S, Kramer U, Ben-Zeev B, et al. Multicenter long- term follow-up of children with idiopathic West syndrome: ACTH versus vigabatrin. Eur J Neurol. 2009;16:482e487. 27. Gaily E, Lommi R, Lapatto R, Lehesjoki A-E. Incidence and outcome of epilepsy syndromes with onset in the first year of life: a retrospective population- based study. Epilepsia. 2016;57:1594e1601. 28. Riikonen R. Long-term outcome of West Syndrome: a study of adults with a history of infantile spasms. Epilepsia. 1996;37:367e372.
Year 2021,
, 149 - 155, 11.03.2021
Abstract
Objective: To determine neurodevelopmental and seizure prognoses in our patients with West syndrome receiving adrenocorticotropic hormone (ACTH) therapy, and to identify the factors affecting these.
Materials methods:
We determined the demographic factors, previous seizure histories, ACTH use and response times, and etiological factors of 34 patients diagnosed with West syndrome in our clinic at 3-24 months and receiving ACTH therapy. We also investigated their neurological development and its effect on seizure prognosis.
Results: We found a significant relationship between patients experiencing seizures before diagnosis and subsequent seizure prognosis. We also found a later response to ACTH and poorer neurodevelopmental and seizure prognoses in patients with symptomatic etiologies. Global developmental delay was determined in 76% of all cases, and seizures persisted despite antiepileptic drugs in 62%.
Conclusions: Symptomatic etiological factors in West syndrome adversely affect the neurodevelopmental process and subsequent seizure prognosis.
References
- 1. Hrachovy RA, Frost JD Jr. Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome). J Clin Neurophysiol 2003;20(06):408–425 2. Swaiman KF, Ashwal S, Ferriero DM, Schor NF, Finkel RS, Gropman LA, Pearl PL, Shevell MI. Swaiman’s pediatric neurology 6th ed. 2017 Elsevier 539-542 3. Kaushik JS, Patra B, Sharma S, Yadav D, Aneja S. Clinical spectrum and treatment outcome of West Syndrome in children from Northern India. Seizure. 2013;22:617–21. 4. Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, et al. Infantile spasms (West syndrome): Update and resources for pediatricians and providers to share with parents. BMC Pediatr. 2012;12:108. 5. Sehgal R, Gulati S, Sapra S, Tripathi M, Kabra M, Pandey RM. Neurodevelopmental and epilepsy outcome in children aged one to five years with infantile spasms—A North Indian cohort. Epilepsy Res. 2014;108:526–34. 6. Riikonen RS. Favourable prognostic factors with infantile spasms. Eur J Paediatr Neurol 2010;14(01):13–18 7. Anlar B ,Yalaz K (1996) Denver II gelişimsel tarama testi Türk çocuklarına uyarlanması ve standardizasyonu el kitabı, Ankara Meteksan Matbası 8. Arzimanoglou A, O’Hare A, Johnston MV, Ouvrier R . Aicardi’s Disease of nervous system in childhood 4th ed. 2018 Mac Keith Press.877-878. 9. Lagae L, Verhelst H, Ceulemans B, De Meirleir L, Nassogne MC, De Borchgrave V, et al. Treatment and long term outcome in West syndrome: The clinical reality. A multicentre follow up study. Seizure. 2010;19:159–64. 10. Yılmaz S, Tekgul H, Serdaroglu G, Akcay A, Gokben S. Evaluation of ten prognostic factors affecting the outcome of West syndrome. Acta Neurol Belg. 2016 Dec;116(4):519-527. 11. Partikian A, Mitchell WG (2010) Neurodevelopmental and epilepsy outcomes in a North American cohort of patients with Infantile spasms. J Child Neurol 25:423–428 12. Araujo AC, Fontenelle LM, Pires L (2011) West syndrome: early remission is not assurance of normal final outcome. Child Care Health Dev 37:261–265 13. Shields WD. Infantile spasms: Little seizures, BIG consequences. Epilepsy Curr. 2006;6:63–9. 14. Riikonen R, Infantile Spasms: Outcome in Clinical Studies . 2020 Feb 4. pii: S0887-8994(20)30041-2 15. Knupp K, Coryel J, Nickels K, et al. Response to treatment in a prospective national infantile spasms cohort. Ann Neurol. 2016;79:475e484. 16. Lux A, Edwards S, Hancock E, et al. The United Kingdom infantile spasm study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomized controlled trial. Lancet. 2004;364:1773e1778. 17. Widjaja E, Go C, McCoy B, Snead OC. Neurodevelopmental outcome of infantile spasms: A systematic review and meta-analysis. Epilepsy Res. 2015 Jan;109:155-62. 18. Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ, et al. Infantile spasms: a U.S. consensus report. . Epilepsia. 2010 Oct;51(10):2175-89. 19. Scheffer I, Berkovic S, Capovilla G, et al. ILAE classification of epilepsies: po- sition paper of the ILEA commission for classification and terminology. Epilepsia. 2017;58:512e521. 20. Riikonen R: Epidemiological data of West syndrome in Finland. Brain Dev 2001, 23:539–541. 21. Trevathan E, Murphy CC, Yeargin-Allsopp M: The descriptive epidemiology of infantile spasms among Atlanta children. Epilepsia 1999, 40:748–751. 22. Webb DW, Fryer AE, Osborne JP: Morbidity associated with tuberous sclerosis: a population study. Dev Med Child Neurol 1996, 38:146–155. 23. Riikonen R (1995) Decreasing perinatal mortality: unchanged infantile spasm morbidity. Dev Med Child Neurol 37:232–238 24. Ibrahim S, Gulab S, Ishaque S, Salem T (2010) Clinical profile and treatment of infantile spasms using vigabatrin and ACTH a developing country perspective. BMC Pediatrics 10:1 25. Mohamed BP, Scott RC, Desai N, Gutta P, Patil S (2011) Seizure outcome in infantile spasms—a retrospective study. Epilepsia 52:746–752 26. Cohen-Sadan S, Kramer U, Ben-Zeev B, et al. Multicenter long- term follow-up of children with idiopathic West syndrome: ACTH versus vigabatrin. Eur J Neurol. 2009;16:482e487. 27. Gaily E, Lommi R, Lapatto R, Lehesjoki A-E. Incidence and outcome of epilepsy syndromes with onset in the first year of life: a retrospective population- based study. Epilepsia. 2016;57:1594e1601. 28. Riikonen R. Long-term outcome of West Syndrome: a study of adults with a history of infantile spasms. Epilepsia. 1996;37:367e372.
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Details
| Primary Language | English |
|---|---|
| Subjects | Health Care Administration |
| Journal Section | Articles |
| Authors | |
| Publication Date | March 11, 2021 |
| Acceptance Date | February 5, 2021 |
| Published in Issue | Year 2021 |