The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study
Yıl: 2021 Cilt: 13 Sayı: 1 Sayfa Aralığı: 149 - 155 Metin Dili: İngilizce DOI: 10.18521/ktd.751363 İndeks Tarihi: 27-06-2021
The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study
Öz: 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.Methods: We determined the demographic factors, previous seizure histories, ACTH use and drug response times, and etiological reasons of 34 patients diagnosed with West syndrome in our clinic at 3-24 months old 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 defined a later response to ACTH and poorer neurodevelopment and seizure prognoses in patients with symptomatic etiologies. Global developmental delay was identified 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.
Anahtar Kelime: West Sendromlu Hastaların Prognostik Değerlendirilmesi: Retrospektif Gözlemsel Çalışma
Öz: Amaç: ACTH tedavisi alan West sendromlu hastalarımızın ileri dönemdekinörogelişimsel prognozu ve nöbet prognozunun saptanması ve bunlara katkı sunanfaktörlerin belirlenmesidir. Gereç ve Yöntem: Kliniğimizde 3-24.aylarında West sendromu tanısı alarak ACTHtedavisi uygulanan 34 hastanın demografik faktörleri, nöbet şekilleri, öncesindeki nöbetöyküleri, ACTH kullanımı ve yanıt süreleri, etyolojik faktörlerini belirledik. Bunlarınnörolojik gelişim ve nöbet prognozuna etkisini araştırdık.Bulgular: Hastaların tanı öncesinde nöbeti olmasının ilerideki nöbet prognozu ileilişkisini anlamlı bulduk. Ayrıca semptomatik etyolojili hastalarda ACTH yanıtını dahageç; nörogelişim ve nöbet prognozunu ise daha kötü saptadık. Tüm hastaların %76’sında global gelişme geriliği, % 62’sinde antiepileptik ilaclara rağmen devam edennöbetler belirledik. Sonuç: West sendromunda semptomatik etyolojik faktörler nörogelişimsel süreci veileriki dönemdeki nöbet prognozunu olumsuz etkilemektedir.
Anahtar Kelime: Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
- 1. Hrachovy RA, Frost JD Jr. Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome). J Clin Neurophysiol 2003;20(06):408–25
- 2. Singhal NS, Harrini C, Sullivan J. Epileptic spasms and myoclonic seizures. In: Swaiman KF, Aswal S, Ferriero DM, eds. Swaiman’s pediatric neurology. Principles and Practice (sixth edition). Elsevier Saunders, UK/USA; 2017. p. 539-42
- 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. Yalaz K, Epir S. The Denver developmental screening test: normative data for Ankara children. Turk J Pediatr. 1983;25(4):245-58
- 8. Arzimanoglou A, Duchowny MS. Epilepsy and other seizure disorders. In: Arzimanoglou A, O’Hare A, Johnston MV, Ouvrier R, editors. Aicardi’s disease of nervous system in childhood 4th ed. Mac Keith Press; 2018. p. 877-8.
- 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;116(4):519-27.
- 11. Partikian A, Mitchell WG. Neurodevelopmental and epilepsy outcomes in a North American cohort of patients with Infantile spasms. J Child Neurol. 2010;25:423-8
- 12. Araujo AC, Fontenelle LM, Pires L. West syndrome: early remission is not assurance of normal final outcome. Child Care Health Dev. 2011;37: 261-5
- 13. Shields WD. Infantile spasms: Little seizures, BIG consequences. Epilepsy Curr. 2006;6:63–9.
- 14. Riikonen R. Infantile spasms: Outcome in clinical studies. Pediatr Neurol. 2020;108:54-64.
- 15. Knupp K, Coryel J, Nickels KC, Ryan N, Leister E, Loddenkemper T, et al. Pediatric Epilepsy Consortium. Response to treatment in a prospective national infantile spasms cohort. Ann Neurol. 2016; 79(3):475-84.
- 16. Lux A, Edwards S, Hancock E, Johnson AL, Kennedy CR, Newton RW, et al. The United Kingdom infantile spasm study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomized controlled trial. Lancet. 2004;364:1773-8.
- 17. Widjaja E, Go C, McCoy B, Snead OC. Neurodevelopmental outcome of infantile spasms: A systematic review and meta-analysis. Epilepsy Res. 2015;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;51(10):2175-89.
- 19. Scheffer I, Berkovic S, Capovilla G, Connolly MB, French J, Guilhoto L, et al. ILAE classification of epilepsies: position paper of the ILEA commission for classification and terminology. Epilepsia. 2017;58(4):512-21.
- 20. Riikonen R. Epidemiological data of West syndrome in Finland. Brain Dev. 2001;23:539–41.
- 21. Trevathan E, Murphy CC, Yeargin-Allsopp M. The descriptive epidemiology of infantile spasms among Atlanta children. Epilepsia. 1999;40:748-51.
- 22. Webb DW, Fryer AE, Osborne JP. Morbidity associated with tuberous sclerosis: a population study. Dev Med Child Neurol. 1996;38:146–55.
- 23. Riikonen R. Decreasing perinatal mortality: unchanged infantile spasm morbidity. Dev Med Child Neurol. 1995;37:232-8
- 24. Ibrahim S, Gulab S, Ishaque S, Salem T. Clinical profile and treatment of infantile spasms using vigabatrin and ACTH a developing country perspective. BMC Pediatrics. 2010;10:1
- 25. Mohamed BP, Scott RC, Desai N, Gutta P, Patil S. Seizure outcome in infantile spasms—a retrospective study. Epilepsia. 2011;52:746-52
- 26. Cohen-Sadan S, Kramer U, Ben-Zeev B, Lahat E, Sahar E, Nevo Y, et al. Multicenter long- term follow-up of children with idiopathic West syndrome: ACTH versus vigabatrin. Eur J Neurol. 2009;16:482-7.
- 27. Gaily E, Lommi R, Lapatto R, Lehesjoki AE. Incidence and outcome of epilepsy syndromes with onset in the first year of life: a retrospective population- based study. Epilepsia. 2016;57(10):1594-601.
- 28. Riikonen R. Long-term outcome of West Syndrome: a study of adults with a history of infantile spasms. Epilepsia. 1996;37:367-72.
| APA | Türay S, hancı f, dilek m, Kabakus N (2021). The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. , 149 - 155. 10.18521/ktd.751363 |
| Chicago | Türay Sevim,hancı fatma,dilek mustafa,Kabakus Nimet The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. (2021): 149 - 155. 10.18521/ktd.751363 |
| MLA | Türay Sevim,hancı fatma,dilek mustafa,Kabakus Nimet The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. , 2021, ss.149 - 155. 10.18521/ktd.751363 |
| AMA | Türay S,hancı f,dilek m,Kabakus N The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. . 2021; 149 - 155. 10.18521/ktd.751363 |
| Vancouver | Türay S,hancı f,dilek m,Kabakus N The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. . 2021; 149 - 155. 10.18521/ktd.751363 |
| IEEE | Türay S,hancı f,dilek m,Kabakus N "The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study." , ss.149 - 155, 2021. 10.18521/ktd.751363 |
| ISNAD | Türay, Sevim vd. "The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study". (2021), 149-155. https://doi.org/10.18521/ktd.751363 |
| APA | Türay S, hancı f, dilek m, Kabakus N (2021). The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. KONURALP TIP DERGİSİ, 13(1), 149 - 155. 10.18521/ktd.751363 |
| Chicago | Türay Sevim,hancı fatma,dilek mustafa,Kabakus Nimet The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. KONURALP TIP DERGİSİ 13, no.1 (2021): 149 - 155. 10.18521/ktd.751363 |
| MLA | Türay Sevim,hancı fatma,dilek mustafa,Kabakus Nimet The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. KONURALP TIP DERGİSİ, vol.13, no.1, 2021, ss.149 - 155. 10.18521/ktd.751363 |
| AMA | Türay S,hancı f,dilek m,Kabakus N The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. KONURALP TIP DERGİSİ. 2021; 13(1): 149 - 155. 10.18521/ktd.751363 |
| Vancouver | Türay S,hancı f,dilek m,Kabakus N The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study. KONURALP TIP DERGİSİ. 2021; 13(1): 149 - 155. 10.18521/ktd.751363 |
| IEEE | Türay S,hancı f,dilek m,Kabakus N "The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study." KONURALP TIP DERGİSİ, 13, ss.149 - 155, 2021. 10.18521/ktd.751363 |
| ISNAD | Türay, Sevim vd. "The Prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study". KONURALP TIP DERGİSİ 13/1 (2021), 149-155. https://doi.org/10.18521/ktd.751363 |
