The evaluation of the neurocognitive development of the symptomatic West Syndrome patients

Esra Sarigecili; Khatuna Makharoblidze; Mustafa Kömür; Cengiz Okuyaz

doi:10.19161/etd.1209083

EN TR

The evaluation of the neurocognitive development of the symptomatic West Syndrome patients

Abstract

Aim: Our study was aimed to determine both demographic and clinical data of patients with symptomatic West Syndrome (WS) and to monitor their neurocognitive development with Bayley Scales of Infant and Toddler Development Screening Test, third edition (Bayley-III) test. Materials and Methods: Fourteen symptomatic WS patients were included in our study. Clinical and demographic data, electroencephalogram (EEG) findings, treatment response, Bayley III developmental test results were recorded before starting the treatment (T0) and in the 12th months of the treatment (T1 and T12). Results: Patients had a significant increase in Bayley-III test scores in all areas at the end of one year (p <0.05). As the patients' EEGs improved, a statistically significant increase was observed in Bayley-III test scores in all areas (p <0.05). However, when the correlation between seizure control and the Bayley-III test scores were evaluated, there was an improvement only in the language area (p< 0,05); but there was no statistically significant difference in other brain areas (p> 0.05). Conclusion: It has been shown that the neurocognitive level gradually improves even in symptomatic type WS with effective treatment during the follow-up of the disease or with the improvement of the EEG findings and seizure control.

Keywords

Semptomatik West Sendromu olan hastaların nörokognitif gelişimlerinin değerlendirilmesi

Öz

Amaç: Çalışmamız semptomatik West Sendromu tanısı olan hastaların hem demografik hem de klinik profillerinin belirlenmesini ve nörokognitif gelişimlerinin Bayley III gelişim testi ile takip edilmesini amaçladı. Gereç ve Yöntem: 14 semptomatik West Sendromu tanısı olan hasta çalışmaya dahil edildi. Klinik ve demografik verileri, elektroensefelogram (EEG) bulguları, tedavi yanıtları, Bayley III gelişimsel test sonuçları tedaviye başlamadan önce (T0) ve 12.ayda (T12) değerlendirildi. Bulgular: Hastaların Bayley III test skorlarında bir yılın sonunda anlamlı bir artış gösterildi (p<0,05). Hastaların EEG’leri düzeldiği zaman Bayley III test sonuçlarında bütün alanlarda anlamlı bir değişim olduğu görüldü (p<0,05). Ancak nöbet kontrolü ve Bayley III test skorları değerlendirildiğinde sadece dil alanında gelişme olduğu görüldü (p<0,05); ancak diğer alanlarda anlamlı bir değişim görülmedi (p>0,05). Sonuç: Uygun tedavi ve nöbet kontrolü ile nörokognitif düzeyin semptomatik west sendromu olan hastalarda bile giderek gelişebildiği gösterildi.

Anahtar Kelimeler

References

Sakakihara Y. Treatment of West syndrome. Brain and Development 2011; 33: 202-06.
Auvin S, Hartman AL, Desnous B, Moreau AC, Alberti C, Delanoe C, et al. Diagnosis delay in West syndrome: misdiagnosis and consequences. Eur J Pediatr 2012; 171: 1695-701.
Riikonen R. Long-term outcome of West syndrome: a study of adults with a history of infantile spasms. Epilepsia 1996; 37: 367-72.
Hrachovy RA, Frost JD. Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome). Journal of Clinical Neurophysiology 2003; 20: 408-25.
Kaya-Kara Ö, Kerem-Günel M, Yiğit Ş. Correlation of the Bayley scales of infant-toddler development-3rd edition and neuro-sensory motor assessment in preterm infants during the first year of life. Turk J Pediatr 2019; 61: 399-406.
Fisher RS. The New Classification of Seizures by the International LeagueAgainst Epilepsy. Curr Neurol Neurosci Rep 2017; 17: 48.
Guveli BT, Cokar O, Dortcan N, Benbir G, Demirbilek V, Dervent A. Long-term outcomes in patients with West syndrome: An outpatient clinical study. Seizure 2015; 25: 68-71.
Yilmaz 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: 519-27.

Nasiri J, Kachuei M, Kermani R, Samaninobandegani Z. Neurodevelopmental outcomes of the West syndrome in pediatric patients: The first report from the Middle-East. Research in Developmental Disabilities 2019; 89: 114-9.
Widjajaa E, Gob C, McCoyb B, Snead C. Neurodevelopmental outcome of infantilespasms: A systematic review andmeta-analysis. Epilepsy Research 2015; 109: 55-162.
Guzzettaa F, Cionib G, Mercuria E, Fazzic E, Biagionid E, Veggiottic P, et al. Neurodevelopmental evolution of West syndrome: A 2-year prospective study. European Journal of Paedıatrıc Neurology 2008; 12: 387-97.
Spennera B, Krois-Neudenberger J, Kurlemannc G, Althausa J, Schwartz O, Fiedler B. The prognostic value of sleep spindles in long-term outcome of West Syndrome. European Journal of Paediatric Neurology 2019; 23: 827-31.
Lux A. Neurodevelopmental status in infantile spasms and West syndrome: the challenge of assessment. Developmental Medicine and Child Neurology 2019; 61: 1242-8.
Pavone P, Striano P, Falsaperla R, Pavone L, Gupta MR et al. Infantile spasms syndrome, West syndrome and related phenotypes: What we know in 2013. Brain and Development 2014; 36: 739-51.
Gupta J, Sharma S, Mukherjee SB, Jain P, Aneja S. Neuro-Developmental and Epilepsy Outcomes of Children with West Syndrome: A Cross-Sectional Study from North India. Ann Indian Acad Neurol 2020; 23: 177-81.
Sharma S, Kaushık, JS, Srıvastava K, Goswamı JN, Sahu JK, Vınayan KP et al. Association of Child Neurology (AOCN) – Indian Epilepsy Society (IES) Consensus Guidelines for the Diagnosis and Management of West Syndrome. Indıan pedıatrıcs 2021;58:54-66.

Details

Primary Language

English

Subjects

Health Care Administration

Journal Section

Research Article

Authors

Esra Sarigecili ^*
0000-0001-8630-4950
Türkiye

Khatuna Makharoblidze
0000-0002-3649-7341
Türkiye

Mustafa Kömür
0000-0001-6453-7323
Türkiye

Cengiz Okuyaz
0000-0001-6391-254X
Türkiye

Publication Date

December 12, 2022

Submission Date

January 19, 2022

Acceptance Date

May 16, 2022

Published in Issue

Year 2022 Volume: 61 Number: 4

DOI

https://doi.org/10.19161/etd.1209083

IZ

https://izlik.org/JA62BM86AU

Cite

RIS / Bibtex

Vancouver

1.Esra Sarigecili, Khatuna Makharoblidze, Mustafa Kömür, Cengiz Okuyaz. The evaluation of the neurocognitive development of the symptomatic West Syndrome patients. EJM. 2022 Dec. 1;61(4):586-92. doi:10.19161/etd.1209083