WOLF-HIRSCHHORN SENDROMU TANILI BİR HASTADA KENDİNE ZARAR VERME DAVRANIŞININ ETKİLİ TEDAVİSİ OLARAK VALPROİK ASİT: OLGU SUNUMU

Year 2025, Volume: 6 Issue: 2, 152 - 156, 20.05.2025

Melike Uysal , Mehmet Ceylan , Selma Tural Hesapçıoğlu

https://doi.org/10.47482/acmr.1605791

Abstract

Wolf-Hirschhorn Sendromu (WHS), sıklıkla uyumsuz davranışlarla birlikte görülen, mikrodelesyonla karakterize nörogelişimsel bir bozukluktur. İlk basamak tıbbi tedavi antipsikotikleri içerir; ancak antipsikotikler yetersiz kaldığında tedavi seçenekleri sınırlı hale gelir. Bu vaka çalışmasında, kendine zarar verme davranışları olan WHS tanılı bir hastada valproik asidin monoterapi olarak uyumsuz davranışları ortadan kaldırabileceği gösterilmek istenmiştir. 10 yaş 4 aylık kadın hasta, yoğun kendine zarar verme davranışları sergilemekteydi. Talepleri karşılanmadığında, başını duvara şiddetle vurup ciddi travmatik yaralanma riski oluşturuyor ve elini ısırıyordu. Daha önce aripiprazol ve risperidon tedavileri yeterli doz ve sürelerde uygulanmış, ancak fayda sağlanamamıştır. Hastanın tedavisi; valproik asit bölünmüş dozlarda 400 mg/gün olacak şekilde düzenlendi. Tedavinin ilk ayından sonra, hasta başını vurma davranışını bıraktı. Ayrıca elini ısırma davranışı da belirgin şekilde azaldı. Başlangıçta 51 puan olarak değerlendirilen Sorun Davranış Kontrol Listesi (ABC) puanı, tedavinin ilk ayından sonra 28 puana düştü. Bu olgu sunumunun hazırlanabilmesi için hastanın ebeveyninden yazılı onam alınmıştır. Bu sonuç, antipsikotiklerin başarısız olduğu nörogelişimsel bozukluk tanılı hastalarda, valproik asidin duygudurum dengeleyici olarak monoterapi şeklinde kendine zarar verme davranışını azaltmada faydalı olabileceğini önermektedir.

Keywords

Nörogelişimsel Bozukluklar , Valproik Asit , Wolf-Hirschhorn Sendromu , Kendine Zarar Verme Davranışı , 4p Delesyonu

VALPROIC ACID AS AN EFFECTIVE TREATMENT FOR SELF-INJURIOUS BEHAVIOR IN A PATIENT WITH WOLF-HIRSCHHORN SYNDROME: A CASE REPORT

Abstract

Wolf-Hirschhorn Syndrome (WHS) is a neurodevelopmental disorder (NDD) characterized by a microdeletion, often accompanied by maladaptive behaviors. First-line medical treatment involves antipsychotics; however, when antipsychotics prove insufficient, treatment options become limited. In this case, it was aimed to demonstrate that valproic acid as monotherapy could eliminate maladaptive behaviors in a patient diagnosed with WHS accompanied by self-injurious behavior. A 10-year 4-month-old female patient with WHS exhibited intense self-injurious behaviors. When her demands were not met, she would bang her head against the wall with significant force, risking severe traumatic injury, and bite her hand. She had previously received sufficient dosage and durations of aripiprazole and risperidone treatments without benefit. Valproic acid was initiated at a dose of 400 mg/day in divided doses. After the first month of treatment, the patient ceased the behavior of banging her head. Additionally, the behavior of biting her hand significantly decreased. The Aberrant Behavior Checklist (ABC) score, initially assessed at 51 points, decreased to 28 points after the first month of treatment. The decrease in the ABC score was particularly marked in the subscales of irritability and social withdrawal.
This outcome suggests that in patients diagnosed with neurodevelopmental disorders (NDD) where antipsychotics have failed, valproic acid as a mood stabilizer may be beneficial as monotherapy in reducing self-injurious behavior.

Keywords

Neurodevelopmental Disorders , Valproic Acid , Wolf-Hirschhorn Syndrome , Self-Injurious Behavior , 4p Deletion

Ethical Statement

Written informed consent was obtained from the patient’s legal guardian for the publication of this report. All procedures were conducted in accordance with the ethical standards of the Declaration of Helsinki.

Supporting Institution

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Thanks

The author would like to acknowledge Dr. Çağlar Uysal from the Department of Mental Health and Diseases, Ankara University, for his contributions during the review process.

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