RETROSPECTIVE ANALYSIS OF WEST SYNDROME ON THE EXAMPLE OF CHILDREN'S CITY CLINICAL HOSPITAL No. 2 ALMATY CITY
Introduction. West syndrome is a rare and severe form of epilepsy that usually affects children in their first year of life. Despite extensive research, the pathogenesis and optimal treatment of this syndrome remain unclear. The optimal approach to the treatment of West syndrome remains a matter of debate, and the efficacy of different treatment strategies varies widely across studies.
Aim. To reveal gender and age characteristics and clinical and electro-radiological features of West's syndrome.
Materials and methods. The study included patients who met the ILAE criteria for the diagnosis of West syndrome.
Data was collected from medical records, including patient demographics, clinical presentation, EEG findings, neuroimaging studies, and treatment outcomes. Descriptive statistics were used to analyze the clinical characteristics of West syndrome in this population. Chi-square tests were used to assess associations between categorical variables. The Kaplan-Meier method was used to estimate time to seizure control, and a log-rank test was used to compare time to seizure control between different treatment groups.
Results. In this retrospective study, cases of West syndrome were analyzed in a children's city clinical hospital over a five-year period. The study aimed to evaluate the clinical characteristics, treatment outcomes, and comorbidities of West syndrome in this population. The results show that early diagnosis and appropriate treatment are critical to achieve favorable outcomes in West syndrome.
Conclusions. The study of clinical, electro-radiological characteristics indicates that the etiological structure is dominated by structurally acquired disorders of the brain, among which the leading etiology is hypoxic-ischemic brain damage due to cerebrovascular accident. In terms of further study of the impact of clinical, electro-radiological and genetic data on outcome using polynomial logistic or multilinear regression.
Bibigul A. Abdygalyk1, https://orcid.org/0000-0001-8171-6112
Marzhan M. Lepesova2, https://orcid.org/0000-0002-4912-4229
Marat R. Rabandiyarov3, https://orcid.org/0000-0003-3471-5478
Bakhytkul D. Myrzalieva2, https://orcid.org/0000-0002-2178-3054
Nazira A. Zharkinbekova4, https://orcid.org/0000-0002-5069-1562
1 Kazakhstan Medical University «Kazakhstan School of Public Health», Almaty c., Republic of Kazakhstan;
2 Kazakh-Russian Medical University, Almaty, Republic of Kazakhstan;
3 Children's City Clinical Hospital No. 2, Almaty, Republic of Kazakhstan;
4 South Kazakhstan Medical Academy, Shymkent c., Republic of Kazakhstan.
1. Adams-Webber T., Riikonen R. Pediatric epilepsy syndromes. Handbook of Clinical Neurology, 2012. 107, 527-541.
2. Chugani H.T., Shields W.D., Shewmon D.A. Infantile spasms: I. Clinical aspects. Epilepsia, 1991. 32 (Suppl 3), S1-S6.
3. Ferrie C.D. Infantile spasms // European Journal of Paediatric Neurology, 2015. 19(2), 127-130.
4. Go C.Y., Mackay M.T. Current understanding of infantile spasms // Canadian Journal of Neurological Sciences, 2013. 40(2), pp. 153-164.
5. Holmes G.L. The pathogenesis of infantile spasms. Mental Retardation and Developmental Disabilities Research Reviews, 2004. 10(1). pp. 25-29.
6. Hrachovy R.A., Frost J.D. The hypsarrhythmia of West syndrome // Epilepsia, 2003. 44 (Suppl 13), pp. 22-30.
7. Hrachovy R.A., Frost J.D., Glaze D.G. A retrospective study of the efficacy of ACTH and prednisone in infantile spasms. Epilepsia, 2013. 54(3). pp. 491-496.
8. Kini L.G., Gee N.R., LaFrance Jr., Cheung R. Pediatric psychogenic non-epileptic seizures: a case report and review of current trends in diagnosis and management // Journal of Child Neurology, 2019. 34(3), pp. 140-146.
9. Lado F.A., Moshé S.L. How do seizures stop? Epilepsia, 2008. 49(10), pp. 1651-1664.
10. Lux A.L., Osborne J.P. A proposal for case definitions and outcome measures in studies of infantile spasms and West syndrome: consensus statement of the West Delphi group // Epilepsia. 2004. 45. pp. 1416–28.
11. Lux A.L. Treatment of infantile spasms. Journal of Child Neurology, 2005. 20(4), pp. 271-277.
12. National Institute for Health and Care Excellence (NICE). Infantile spasms: recognition, referral and diagnosis. 2021. Retrieved from https://www.nice.org.uk/guidance/ng204 (Accessed 27 April 2022).
13. O'Callaghan F.J., Lux A.L., Darke K., Edwards S.W., Hancock E., Johnson A.L., Sander J.W. Outcome for patients with infantile spasms treated in the United Kingdom over a decade // Epilepsia, 2000. 41(3), pp. 365-371.
14. Oht А.S., Ohtsuka Y. Electroencephalographic and clinical predictors of the outcome of treatment of infantile spasms with high-dose adrenocorticotropic hormone // Brain and Development, 2001. 23(7), pp. 674-678.
15. Osawa M., Kondo T. Treatment of infantile spasms // Brain and Development, 2013. 35(1), pp. 7-22.
16. Pavone P., Praticò A.D., Ruggieri M., Falsaperla R. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2019. Brain Sciences, 2019. 9(10), 267р.
17. Pellock J.M., Hrachovy R., Shinnar S., Baram T.Z., Bettis D., Dlugos D.J., Nordli D.R. Infantile spasms: a U.S. consensus report // Epilepsia, 2010. 51(10), pp. 2175-2189.
18. Riikonen R. Infantile spasms: therapy and outcome // Journal of Child Neurology, 2009. 24(8), pp. 1204-1208.
19. Riikonen R. Recent advances in the pharmacotherapy of infantile spasms // CNS Drugs, 2011. 25(3), 199-216.
20. Sameer M.Z., Wirrell E., Yozawitz E. et al. ILAE classification and definition of epilepsy syndromes with onset in neonates and infants: Position statement by the ILAE Task Force on Nosology and Definitions // Journal Epilepsia, 2022, 1–49, First published 03 May 2022.
21. Watanabe K., Negoro T., Okumura A., Hayakawa F. Characteristics and long-term outcome of 60 patients with West syndrome // Brain and Development, 2010. 32(7), pp. 566-573.
22. West W.J. On convulsive diseases in infancy // The Medical Times, 1841. 3, 35-38.
Number of Views: 65
Category of articles:
Original articles
Bibliography link
Abdygalyk B.A., Lepesova M.M., Rabandiyarov M.R., Myrzalieva B.D., Zharkinbekova N.A. Retrospective analysis of West syndrome on the example of Children's City Clinical Hospital No. 2, Almaty city // Nauka i Zdravookhranenie [Science & Healthcare]. 2023, (Vol.25) 3, pp. 137-143. doi 10.34689/SH.2023.25.3.018Related publications:
ANALYSIS OF CLINICAL AND DEMOGRAPHIC INDICATORS OF PATIENTS WITH PARKINSON'S DISEASE IN THE SOUTHERN REGION OF KAZAKHSTAN
A COMPARATIVE STUDY OF DRUG POLICY REGARDING ORPHAN DISEASES IN THE USA, THE EUROPEAN UNION, AND THE REPUBLIC OF KAZAKHSTAN
MUTATIONS IN CARDIAC ION CHANNEL GENES IN KAZAKHSTANI PATIENTS WITH LONG QT SYNDROME
GENETIC FEATURES OF CONGENITAL DYSFUNCTION OF THE ADRENAL CORTEX CAUSED BY 21-HYDROXYLASE DEFICIENCY IN PERSONS OF KAZAKH NATIONALITY
NON-MOTOR SYMPTOMS IN PARKINSON'S DISEASE USING THE EXAMPLE OF AKTOBE PATIENTS