Pseudotumor cerebri in children: etiology, clinical findings, prognosis

Year 2019, Volume: 44 Issue: 2, 447 - 452, 30.06.2019

Gülen Gül Mert , Neslihan Özcan Şeyda Beşen Kemal Yar Özlem Hergüner , Faruk İncecik , Şakir Altunbaşak

https://doi.org/10.17826/cumj.454987

Abstract

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

Çocuklarda psödotümör serebri: etyoloji, klinik bulgular, prognoz

Abstract

Amaç: Psödotümör serebri tanısı alan hastaların klinik ve nörogörüntüleme bulguları, etiyolojileri, tedavi şekilleri ve süreleri, tedaviye yanıtları ve nörolojik sekel varlıkları gözden geçirildi

Gereç 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.

Keywords

Idiopathic intracranial hypertension, papillae edema, headache, childhood

References

• 1) Digre KB, Bruce BB, McDermott MP, et al. Quality of life in idiopathic intracranial hypertension at diagnosis: IIH Treatment Trial results. Neurology 2015;84:2449
• 2) Dessardo NS, Dessardo S, Sasso A, Sarunic AV, Dezulovic MS. Pediatric idiopathic intracranial hypertension: clinical and demographic features. Coll Antropol. 2010;34(Suppl 2):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. https://doi.org/10.1016/j.pediatrneurol.2017.04.021
• 5) Parunyou Julayanont, Amputch Karukote, Doungporn Ruthirago, et al. Idiopathic intracranial hypertension: Ongoing clinical challenges and future prospects. Journal of Pain Research 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(8):461–74. https://doi.org/10.1001/archneur.1982.00510200003001. 7) Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81:1159-1165.
• 8) McGeeney BE, Friedman DI. Pseudotumor cerebri pathophysiology. Headache 2014;54:445-458.
• 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;159:344-352.
• 11) Amira Masri, Amani Jaafar, Rasha Noman, et al. Intracranial Hypertension in Children: Etiologies, Clinical Features, and Outcome. Journal of Child Neurology 2015, Vol. 30(12) 1562-1568.
• 12) Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophthalmology 1998; 105:1686.
• 13) Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011; 32:1986.
• 14) Hoffmann J, Huppertz HJ, Schmidt C, et al. Morphometric and volumetric MRI changes in idiopathic intracranial hypertension. Cephalalgia 2013; 33:1075
• 15) Aylward SC, Aronowitz C, Reem R, Rogers D, Roach ES. Intracranial hypertension without headache in children. J Child Neurol. 2015;30(6):703–6. https://doi.org/10.1177/ 0883073814540522.
• 16) Aylward SC, Way AL. Pediatric Intracranial Hypertension: a Current Literature Review. Current Pain and Headache Reports (2018) 22:14. https://doi.org/10.1007/s11916-018-0665-9
• 17) Ana B. Chelse, and Leon G. Epstein. Intracranial Hypertension in Children without Papilledema. Pediatric Neurology Briefs 2015;29(8):6.
• 18) Lee YA, Tomsak RL, Sadikovic Z, Bahl R, Sivaswamy L. Use of ocular coherence tomography in children with idiopathic intracranial hypertension—a single-center experience. Pediatr Neurol. 2016;58:101–6 e1.https://doi.org/10.1016/j.pediatrneurol.2015. 10.022.
• 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(18):1564–7. https://doi. org/10.1212/WNL.0b013e3182190f51.
• 20) Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiopathic intracranial hypertension. J Neurol Neurosurg Psychiatry 2012; 83:488.
• 21) NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Wall M, McDermott MP, 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.
• 22)Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989; 46:1049.
• 23)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(5):322–7. https:// doi.org/10.1111/j.1600-0404.2007.00905.x
• 24) Finsterer J, Földy D, Fertl E. Topiramate resolves headache from pseudotumor cerebri. J Pain Symptom Manage 2006; 32:401.
• 25) Nils Becker; Golo Kronenberg, Lutz Harms, Edzard Wiener, et al. Rapid Improvement of Olfaction After Lumbar Puncture in a Patient With Idiopathic Intracranial Hypertension. Headache 2016;56:890-892.