Gülen GÜL MERT, Neslihan ÖZCAN, Şeyda BEŞEN, Kemal YAR, Özlem HERGÜNER, Faruk İNCECİK, Şakir ALTUNBAŞAK

Çocuklarda psödotümör serebri: etyoloji, klinik bulgular, prognoz

Amaç: Psödotümör serebri tanısı alan hastaların klinik ve nörogörüntüleme bulguları, etiyolojileri, tedavi şekilleri ve süreleri, tedaviye yanıtları ve nörolojik sekel varlıkları gözden geçirildiGereç ve Yöntem: Çalışmaya dahil edilen hastalar Haziran 2015 ile Mayıs 2018 arasında Çukurova Tıp Fakültesi Çocuk Nöroloji Bilim Dalında takip edildi. Yirmi yedi hastanın yaş, cinsiyet dağılımı, antropometrik ölçümleri, BOS basınçları, nörolojik, oftalmolojik, nörogörüntüleme, nörolojik sekel bulguları, tedavi modaliteleri ve süreleri, tedavi yanıtı retrospektif olarak incelendi. Bulgular: BOS basıncı ortalaması 43,29,1cmH2O idi. Etiyolojide en sık neden obezite (%33,3), demir eksikliği anemisi (%18,5) ve venöz sinüs trombozu (%14,8) idi. Hastaların %59,3’üne yalnızca asetazolamid tedavisi verildi, %25,9’una topiramat eklendi, %14,8’ine steroid verildi. 25 hastanın nörolojik ve oftalmolojik bulgularında medikal tedavi ile tamamen düzelme görüldü.Sonuç: Psödotümör serebri hastalığında en sık oluşan nörolojik sekel kalıcı görme kaybıdır. Bu durum geri dönüşümsüz olur ve çocuğun tüm hayatını etkiler. Bu nedenle başağrısı, görme kaybı, çift görme şikayetleri olan çocuklarda Psödotümör serebri tanısının düşünülmesi gerektiğinin bilinmesi ve çocukların hızlı ve uygun şekilde tedavisi prognozda oldukça önemlidir.

Anahtar Kelimeler:

Psödotümor serebri, papil ödem

Pseudotumor cerebri in children: etiology, clinical findings, prognosis

Purpose: Clinical and neuroimaging findings, aetiologies, treatment modalities and durations, response to treatment, and neurological sequelae of the patients diagnosed with pseudotumor cerebri were reviewed.Materials and Methods: A total of 27 patients who followed up in the Department of Pediatric Neurology at Çukurova Medical Faculty between June 2015 and May 2018 were included in this study. Age, gender distribution, anthropometric measurements, cerebrospinal fluid pressures, neurological, ophthalmologic, neuroimaging and neurological sequelae findings, treatment modalities and durations, response to therapy of 27 patients were reviewed retrospectively. Results: Mean cerebrospinal fluid pressure was 43.29.1cmH2O. The most frequent cause in aetiology were obesity (33.3%), iron deficiency anemia (18.5%) and venous sinus thrombosis (14.8%). All patients were treated with acetazolamid, 59.3% patients received only acetazolamid and 25.9% of patients received combined therapy with acetazolamide and topiramate and 14.8% of patients received combined therapy with acetazolamide and steroids. Of the patients, 25 had excellent neurological and ophtalmological outcome with medical treatment. Conclusion: The most frequently-seen neurological sequelae in pseudotumor cerebri is permanent visual impairment This irreversible situation affects the whole life of child. Therefore it is quite important to think about the diagnosis of pseudotumor cerebri for the children with complaints of headache, visual impairment and diplopia, and to subject them to treatment quickly and properly.

Keywords:

Idiopathic intracranial hypertension, papillae edema headache, childhood,

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1. Digre KB, Bruce BB, McDermott MP, Galetta KM, Balcer LJ, Wall M. Quality of life in idiopathic intracranial hypertension at diagnosis: IIH Treatment Trial results. Neurology. 2015;84:2449-56.
2. Dessardo NS, Dessardo S, Sasso A, Sarunic AV, Dezulovic MS. Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol. 2010;34:217–21.
3. Aylward SC, Waslo SC, Johnathan N, Tanne E. Manifestations of Pediatric Intracranial Hypertension from the Intracranial Hypertension Registry. Ped Neurol. 2016;1-7.
4. Gillson N, Jones C, Reem RE, Rogers DL, Zumberge N, Aylward SC. Incidence and demographics of pediatric intracranial hypertension. Pediatr Neurol. 2017;73:42–7.
5. Julayanont P, Karukote A, Ruthirago D, Panikkath D, Panikkath R. Idiopathic intracranial hypertension: Ongoing clinical challenges and future prospects. J Pain Res. 2016;9:87–99.
6. Corbett JJ, Savino PJ, Thompson HS, Kansu T, Schatz NJ, Orr LS et al. Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss. Arch Neurol. 1982;39:461-74.
7. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81:1159-65.
8. McGeeney BE, Friedman DI. Pseudotumor cerebri pathophysiology. Headache. 2014;54:445-58.
9. Hainline C, Rucker JC, Balcer LJ. Current concepts in pseudotumor cerebri. Curr Opin Neurol. 2016;29:84- 93.
10. Paley GL, Sheldon CA, Burrows EK, Chilutti MR, Liu GT, McCormack SE. Overweight and obesity in pediatric secondary pseudotumor cerebri syndrome. Am J Ophthalmol. 2015;159:344-52.
11. Masri A, Jaafar A, Noman R, Gharaibeh A, Ababneh OH. Intracranial hypertension in children: etiologies, clinical features, and outcome. J Child Neurol. 2015;30:1562-8.
12. Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology. 1998;105:1686-90.
13. Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol. 2011;32:1986-93.
14. Hoffmann J, Huppertz HJ, Schmidt C, Kunte H, Harms L, Klingebiel R et al. Morphometric and volumetric MRI changes in idiopathic intracranial hypertension. Cephalalgia 2013;33:1075-84.
15. Aylward SC, Aronowitz C, Reem R, Rogers D, Roach ES. Intracranial hypertension without headache in children. J Child Neurol. 2015;30:703–6.
16. Aylward SC, Way AL. Pediatric intracranial hypertension: a current literature review. Curr Pain Headache Rep. 2018;22:14.
17. Chelse AB, Epstein LG. Intracranial hypertension in children without papilledema. Pediatr Neurol Briefs. 2015;29:61-2.
18. Lee YA, Tomsak RL, Sadikovic Z, Bahl R, Sivaswamy L. Use of ocular coherence tomography in children with idiopathic intracranial hypertension a singlecenter experience. Pediatr Neurol. 2016;58:101–6.
19. Ko MW, Chang SC, Ridha MA, Ney JJ, Ali TF, Friedman DI et al.Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study. Neurology. 2011;76:1564–7.
20. Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry. 2012;83:488-94.
21. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Wall M, McDermott, Kleburtz KD, Corbett JJ, Feldon SE, Friedman DI et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014;311:1641- 51.
22. Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol. 1989;46:1049-51.
23. İncecik F, Hergüner Ö, Altunbaşak Ş. Evaluation of sixteen children with pseudotumor cerebri. Turk J Pediatr. 2011;53:55-8.
24. Celebisoy N, Gokcay F, Sirin H, Akyurekli O. Treatment of idiopathic intracranial hypertension: topiramate vs acetazolamide, an open-label study. Acta Neurol Scand. 2007;116:322–7.
25. Finsterer J, Földy D, Fertl E. Topiramate resolves headache from pseudotumor cerebri. J Pain Symptom Manage. 2006;32:401-2.
26. Becker N, Kronenberg G, Harms L, Wiener E, Kreutz K, Eltiri A. et al. Rapid improvement of olfaction after lumbar puncture in a patient with idiopathic intracranial hypertension. Headache. 2016;56:890-2.