Pseudotumor cerebri syndrome in childhood: incidence, clinical profile and risk factors in a national prospective population-based cohort study

Abstract

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

Update in Pediatric Pseudotumor Cerebri Syndrome

“…Puberty is the threshold between the "adult" phenotype of obese and female from a "prepubertal phenotype, in which the sex distribution is equal in children with normal to thin body mass index (BMI). 1,4,[24][25][26] A recent study further stratified pediatric cohorts, showing that young children (females <7 years old and boys <8.5 years old) tend to be of normal height and weight; early adolescents are typically taller and overweight; lastly, a late adolescent group that is similar to the typical PTCS adult are usually obese with normal height. 27 One study found 82% of children with PTCS were obese in the 12-to 15-year-old cohort.…”

“…27 One study found 82% of children with PTCS were obese in the 12-to 15-year-old cohort. 1 There are conflicting studies explaining the anthropometrics of secondary pediatric PTCS. In one study of children with secondary PTCS, the children were obese 28 ; however, previous studies showed that the majority of children with minocyclineassociated secondary PTCS, 29 with exposure to recombinant growth hormone 30,31 or aldosteronism, 32 were not obese at presentation.…”

“…Internationally, the incidence of pediatric PTCS is similar and reported as 0.60 to 0.71 per 100,000 children. [1][2][3] The incidence increases with age. 1 The primary symptoms in children are vision loss and headache.…”

“…[1][2][3] The incidence increases with age. 1 The primary symptoms in children are vision loss and headache. Older children can describe double vision, tinnitus, and transient vision loss; however, asymptomatic or nonspecific findings such as apathy and irritability can be present in toddlers and young children.…”

Idiopathic Intracranial Hypertension in Children and Adolescents: An Update

“…The exact prevalence of PTC in the pediatric population is not well established. Recent studies from the UK using the Friedman criteria found an annual incidence of 0.71 per 100,000. Age specific incidences increases from 0.17 to 0.75 to 1.32 per 100,000 in patients aged 1‐6, 7‐11, and 12‐16 years, respectively.…”