Patients with pseudotumor cerebra demographic data, imaging findings and evaluation of the treatment

Introduction-Purpose: Pseudotumor cerebri syndrome (PSS) is defined as a clinical picture with normal cerebrospinal fluid (CSF) protein and cell content, without intracranial space-occupying lesion, infection, hydrocephalus, venticulomegaly, and signs and symptoms of increased intracranial pressure syndrome. The aim of this study is to explain the etiology, clinical presentation, examinations, management and results of children diagnosed with Pseudotumor cerebri, who were followed up in Pamukkale University Faculty of Medicine, Pediatric Neurology Clinic between 2018 and 2022, and describe the factors associated with improvement in papilledema, visual symptoms and headache. Material-Method: Pamukkale University Non-Interventional Clinical Research Ethics Committee approval (E-60116787-020-293710/15.11.2022) was obtained for the study. The data of 46 patients younger than 18 years of age who were followed up with the diagnosis of PTS according to the modified Dandy criteria in Pamukkale University Faculty of Medicine, Department of Pediatric Neurology between September 2018 and September 2022 were evaluated. Patient data were obtained by retrospective scanning of patient files. According to the 2013 Friedman diagnostic criteria, patients with a definite and probable diagnosis were determined. The patients were divided into two groups as primary PTC and secondary PTC as a result of the combination of many factors. Patients younger than 11 years of age were considered prepubertal and above pubertal. Those with a BMI above 30 were considered obese. Those with Frisen grade 1 or 2 were categorized as "mild papilledema", Frisen 3 or "optic nerve head elevation" as "moderate papilledema", and Frisen 4 or 5 as "severe papilledema". Discussion-Conclusions: The mean age at presentation was 11.3 ± 4.5 years, and 30.5% of the patients were in the prepubertal age group and 69.5% in the postpubertal age group. The obesity rate was significantly lower in the prepubertal period than in the postpubertal period (p<0.05). The most common symptoms were papilledema, headache, transient visual disturbances, and nausea and vomiting, respectively. Statistically significant headache complaints were found in obese patients compared to normal weight patients (p<0.05). When the CSF opening pressures were compared between the primary/secondary and prepubertal/pubertal groups, there was no significant difference (p>0.05). The rate of the cases that responded to only acetazolamide treatment was 67.3%, and additional topiramate treatment was needed in the remaining patients. The dose of acetazolamide in the treatment was significantly higher in patients with papilledema compared to patients without (p<0.05). Acetazolamide side effects were found to be significantly lower in the primary group than in the secondary group (p<0.05). Optic nerve sheath fenestration (ONSF) was performed in seven patients, optic nerve sheath fenestration and lumbo-peritoneal (LP) shunt in one patient, and cerebral venous sinus stenting in one patient. Visual loss developed in 6.5% (n=3) during follow-up.