Çocukluk Çağı Psödotümör Serebrili Hastaların Retrospektif Değerlendirmesi: Tek Merkez Deneyimi

Amaç: Psödotümör serebri intrakraniyal basınç artışı bulgularının gözlendiği, kafa içi yer kaplayan patolojilerin bulunmadığı nadir nörolojik bir durumdur. Çalışmamızın amacı psödotümör serebri tanısıyla takip edilen hastaların demografik özellikleri, tanı, tedavi ve prognozlarının tespit edilmesidir. Gereç ve Yöntem: Üçüncü basamak eğitim ve araştırma hastanesi çocuk nöroloji polikliniğinde Eylül 2017 - Mart 2019 tarihleri arasında psödotümör serebri tanısı ile takip edilen 15 hastanın (9 kız, 6 erkek) verileri retrospektif olarak değerlendirildi. Bulgular: Hastaların ortalama yaşı 10,26±4,75 yıl (aralık: 1-16 yıl) idi. İlk başvuru sırasında en sık belirti ve bulgular; papilödem, baş ağrısı, bulantı- kusma, halsizlik, bulanık görme ve çift görmeydi. Tüm hastalara beyin manyetik rezonans görüntüleme (MRG) ve 6/15 hastaya beyin MRG venografi uygulandı, üç hastada kafa içi basınç artışını destekleyen bulgular saptandı. Beyin omurilik sıvısı giriş basıncı ortalama 306±75 mmH2O (aralık: 260- 470 mmH2O) idi. Etiyolojik olarak büyüme hormonu kullanımı (n=2), Down sendromu (n=1), Guillain-Barre sendromu (n=1), ailesel Akdeniz ateşi (n=1), Chiari malformasyonu tip 1 (n=1), akut lenfoblastik lösemi (n=1), A vitamini eksikliği (n=1), obezite ve MODY tip 3 (n=1) tespit edildi. Tüm hastalara başlangıç tedavisi olarak asetazolamid başlandı. Ortalama 7,87±5,01 ay (aralık: 2-15 ay) takip süresi içerisinde 9/15 hastada tam iyileşme, 5/15 hastada papilödem bulgularında belirgin iyileşme, 1/15 hastada kısmi iyileşme saptandı. Hastaların hiçbirinde kalıcı görme kaybı veya görme alanı defekti gelişmedi. Tedavi kesimi sonrası veya tedavi sırasında hiçbir hastada nüks izlenmedi. Sonuç: Psödotümör serebri çocukluk çağında özellikle postpubertal dönemde daha sık görülür. Obezite çocukluk çağında nadir nedenlerdendir. Pediyatrik hastaların yarısından fazlasında psödotümör serebriye neden olabilen etiyolojik bir neden tespit edilebilir ve çalışmamızda en sık neden olarak büyüme hormonu kullanımı belirlendi. Psödotümör serebri uzun süreli tedavi ve takip gerektiren nadir bir hastalıktır, ancak erken tanı alan, uygun tedavi gören ve tedaviye iyi uyum gösteren hastalarda genellikle prognoz iyidir.

Retrospective Evaluation of Children With Pseudotumor Cerebri: A Single Center Experience

Objectives: Pseudotumor cerebri is a rare neurological condition in which signs of increased intracranial pressure are observed and there are no intracranial space-occupying pathologies. The aim of our study is to determine the demographic characteristics, diagnosis, treatments and prognosis of patients with pseudotumor cerebri. Materials and Methods: The data of 15 patients (9 girls, 6 boys) who were followed up with the diagnosis of pseudotumor cerebri between September 2017 and March 2019 in a tertiary training and research hospital pediatric neurology outpatient clinic were retrospectively evaluated. Results: The mean age of the patients was 10.26±4.75 years (range: 1-16 years). The most common signs and symptoms during the first admission were papilledema , headache, nausea-vomiting, weakness, blurred vision and double vision. Brain magnetic resonance imaging (MRI) was performed in all patients. Supporting findings of increased intracranial pressure were detected in three patients. The mean cerebrospinal fluid inlet pressure was 306±75 mmH2O (range: 260-470 mmH2O). Etiologically, the use of growth hormone (n=2), Down syndrome (n=1), Guillain-Barré syndrome (n=1), familial Mediterranean fever (n=1), Chiari malformation type 1 (n=1), acute lymphoblastic leukemia (n=1), vitamin A deficiency (n=1), obesity (n=1) and MODY tip 3 (n=1) were detected. Acetazolamide was started in all patients as initial therapy. Nine of 15 patients had full recovery, five of 15 patients had significant improvement in papilledema findings, and one of 15 patients had partial improvement. None of the patients developed permanent vision loss or visual field defect. No recurrence was observed in any patient after treatment discontinuation or during treatment. Conclusion: Pseudotumor cerebri is more common in childhood, especially in the postpubertal period. Obesity is a rare cause in childhood. An etiological cause that may cause pseudotumor cerebri can be detected in more than half of the pediatric patients, and the use of growth hormone was the most common in our study. Pseudotumor cerebri is a rare disease that requires long-term treatment and follow-up, but the outcome is generally well in patients who are diagnosed early, receive particular treatment and comply well with treatment.

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