West Sendromlu Hastaların Prognostik Değerlendirilmesi: Retrospektif Gözlemsel Çalışma

Year 2021, Volume: 13 Issue: 1, 149 - 155, 11.03.2021

Sevim Türay Fatma Hancı Mustafa Dilek Nimet Kabakuş

https://doi.org/10.18521/ktd.751363

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.

Keywords

West Sendromu, ACTH Tedavisi, Spazm, Nörogelişimsel Gerilik, Prognoz

The prognostic Evaluation of West Syndrome Patients: A Retrospective Observational Study

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.

Keywords

West syndrome, ACTH therapy, spasm, neurodevelopmental delay, prognosis

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