Etiology and prognosis of childhood pseudotumor cerebri syndrome: A retrospective single center study from Turkey

Abstract

Objective: This study aim to investigate children who were diagnosed with pseudotumor cerebri (PTC) from a tertiary hospital in Turkey.

Methods: This was a retrospective study based on medical records of 16 children diagnosed with PTC. All patients were followed up in the Pediatric Neurology Department of Keçiören Training and Research Hospital, between 2017 and 2021. Clinical and laboratory features of patients were reviewed.

Results: Among the 16 patients diagnosed with pseudotumor cerebri, 50% were girls and 50% boys. The mean age at the disease onset was 12± 3.8 years (range: 4-17 years). Eleven patients (68.8%) were in the pubertal period and, five (31.3%) of them were in pre-pubertal period. Five patients (31.3%) were diagnosed with primary and eleven (68.8%) patients with secondary PTC. Underlying etiologies were obesity (37.5%), sinusitis (37.5%), and systemic lupus erythematosus (6.3%). Laboratory findings revealed vitamin D deficiency (68.8%); vitamin B12 deficiency (25%), iron deficiency anemia (12.5%), and vitamin A deficiency (6.3%). The most common symptoms were headache (93.8%), nausea and vomiting (25%), blurred vision (18.8%), photophobia (18.8%). All patients had bilateral papilledema. Four children were asymptomatic with papilledema detected incidentally on routine eye examination. Nine patients received medical treatment. Acetazolamide was the most prescribed medication (56%). Only one patient with epilepsy had combined treatment with topiramate, acetazolamide and then lumboperitoneal shunt. All patients responded to treatment with full recovery.

Conclusion: PTC is not rare in children, most were pubertal, and a quarter were asymptomatic diagnosed in routine fundus examination.