Primary pseudotumor cerebri in association with obesity - a rare differential diagnosis even in childhood

Abstract

Aims: Primary pseudotumor cerebri (PTC) is defined as elevated cerebrospinal fluid (CSF) pressure of more than 20cm H2O and exclusion of infectious, structural and systemic causes. Without treatment PTC can lead to loss of visual field and acuity. Case report 1: 13 year old, prepubescent, obese boy (BMI 33.5; 10>P97); since 10d headache and neck pain, vomiting; papilledema and loss of visual field. CSF pressure 80cm H2O. Exclusion of potential infectious, structural and systemic causes. Under treatment with acetazolamide, repeated lumbar puncture and gain of weight symptoms resolved. Case report 2: 15 year old, postpubertal, obese girl (BMI25; P97). Since one year intermittent headache, nausea; papilledema and slight affection of visual field. Intake of oral contraceptives. CSF pressure 37cm H2O. Exclusion of potential infectious, structural and systemic causes. Under treatment with acetazolamide symptoms resolved. Discussion: Elevated CSF pressure and exclusion of infectious, structural and systemic causes is called primary or idiopathic pseudotumor cerebri (PTC). In adults there is a strong association with obesity and a predilection for women. Contrary to adults obesity in association with PTC in childhood is reported to be uncommon. Structural and systemic causes play a stronger role like cranial venous outflow abnormalities (e.g. venous sinus thrombosis), medications, endocrine abnormalities, hypovitaminosis (vitamin A/D), anemias and autoimmune disorders. None the less our reported children show that non-typical patterns (1) or even less severe symptoms (2) in association with obesity should remind us of the differential diagnosis of PTC – especially on the background of the increasing incidence of obesity.