Aim To investigate the epidemiology, clinical profile and risk factors of pseudotumor cerebri syndrome (PTCS) in children aged 1-16 years.Methods A national prospective population-based cohort study over 25 months. Newly diagnosed PTCS cases notified via British Paediatric Surveillance Unit (BPSU) were ascertained using classical diagnostic criteria and categorised according to 2013 revised diagnostic criteria. We derived national age, sex and weight-specific annual incidence rates and assessed effects of sex and weight category. ResultsWe identified 185 PTCS cases of which 166 also fulfilled revised diagnostic criteria. The national annual incidence (95% CI) of childhood PTCS aged 1-16 years was 0.71 (0.57-0.87) per 100,000 population increasing with age and weight to 4.18 and 10.7 per 100,000 in obese 12-15 year old boys and girls respectively. Incidence rates under 7 years were similar in both sexes. From 7 years onwards, the incidence in girls was double that in boys, but only in overweight (including obese) children. In 12-15 year old children, an estimated 82% of the incidence of PTCS was attributable to obesity. Two subgroups of PTCS were apparent: 168 (91%) cases aged from 7 years frequently presented on medication and with headache, and were predominantly female and obese. The remaining 17 (9%) cases under 7 years often lacked these risk factors and commonly presented with paralytic squint. ConclusionsThis uniquely largest population-based study of childhood PTCS will inform the design of future intervention studies. It suggests that weight reduction is central to the prevention of PTCS.3

Aims IIH is the rare condition of intracranial hypertension without identifiable pathology. Associated headache and procedures to treat it can be distressing and the papilloedema has an associated risk of blindness. Previous childhood IIH epidemiological data was limited to hospital-based retrospective case series. This study reports a contemporary national annual incidence of childhood IIH, associated risk factor of obesity, current management and outcome at one-year following diagnosis. Methods Monthly notification for 25 months from July 2007 of newly diagnosed IIH cases aged 1-16 years through British Paediatric Surveillance Unit reporting scheme by all reporting UK paediatricians. One-year post IIH diagnosis outcome data was collated. The IIH case definition comprised: At least two key features: Symptoms of raised intracranial pressure and/or visual symptoms; Papilloedema; Opening cerebrospinal fluid pressure (CSF) above 20 cm H2O; and all essential criteria; Normal consciousness level; CT/MRI brain±MR/CT venography does not reveal a structural cause such as ventricular dilatation, cerebral or vascular lesion, venous sinus thrombosis; Normal CSF contents; Results 379 notifications identified 185 (93-confirmed, 92-probable) cases of childhood IIH. 85% (158/185) submitted one-year post diagnosis data. Median age at diagnosis was 12 years. 68% (125/185) were female. 60% (105/174) were obese (BMI ≥ 98th centile). Within this cohort, 81% (150/185) received medical treatment (s), 19% (35/185) had 3 or more lumbar punctures and 13% (24/185) underwent neurosurgery. Visual acuity and/or field deficits were present in 33% (61/185) at diagnosis and 10% (16/158) at one-year follow up. Estimated UK annual incidence is 0.8 (1.0 for girls, 0.5 for boys) per 100,000 child population aged 1-16 years. Conclusions An average of 60 girls and 29 boys were diagnosed with childhood IIH each year in the UK. Obesity is the commonest association. The majority received medical treatment (s), a reasonable proportion needed repeated lumbar punctures and neurosurgery. Despite interventions one-in-ten had reported visual deficits at one-year post IIH diagnosis. This study highlights the urgent need to generate well-designed multicentre controlled trials to inform the efficacy of current non-evidenced based medical and surgical treatments in childhood IIH.

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