Article Text

other Versions

PDF
Pseudotumor cerebri syndrome in childhood: incidence, clinical profile and risk factors in a national prospective population-based cohort study
  1. Yim-Yee Matthews1,
  2. Fiona Dean2,
  3. Ming J Lim3,
  4. Karen Mclachlan4,
  5. Alan S Rigby5,
  6. Guirish A Solanki6,
  7. Catharine P White7,
  8. William P Whitehouse8,
  9. Colin R Kennedy9
  1. 1Paediatric Department, Wrexham Maelor Hospital, Wrexham, UK
  2. 2Ophthalmology Department, University Hospital Coventry and Warwickshire NHS Trust, Coventry, UK
  3. 3Department of Children's Neurosciences, Evelina London Children's Hospital, Guy's and St Thomas’ NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London, UK
  4. 4Paediatric Department, University Hospital Coventry and Warwickshire NHS Trust, Coventry, UK
  5. 5Division of Cardiovascular and Respiratory Medicine, University of Hull, Hull, UK
  6. 6Department of Paediatric Neurosurgery, Birmingham Children's Hospital, University of Birmingham, Birmingham, UK
  7. 7Department of Child Health, Morriston Hospital, Swansea, UK
  8. 8Department of Paediatric Neurology, School of Medicine, University of Nottingham, Nottingham University Hospitals NHS Trust, Nottingham, UK
  9. 9Clinical Neurosciences, Faculty of Medicine, University of Southampton, University Hospital Southampton, Southampton, UK
  1. Correspondence to Dr Yim-Yee Matthews, Paediatric Department, Wrexham Maelor Hospital, Croesnewydd Road, Wrexham LL13 7TD, UK; yim-yee.matthews{at}wales.nhs.uk

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 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 categories.

Results We identified 185 PTCS cases of which 166 also fulfilled revised diagnostic criteria. The national annual incidence (95% CI) of PTCS in children aged 1–16 years was 0.71 (0.57 to 0.87) per 100 000 population increasing with age and weight to 4.18 and 10.7 per 100 000 in obese boys and girls aged 12–15 years, 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 children aged 12–15 years, 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.

Conclusions This uniquely large 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.

  • pseudotumor cerebri
  • idiopathic intracranial hypertension
  • Obesity
  • Epidemiology
  • BPSU

Statistics from Altmetric.com

Footnotes

  • Contributors The surveillance study was designed by Y-YM with key input from KMc and FD in the development of study protocol and survey questionnaires. WPW, CRK and CPW provided oversight of the surveillance study conducted by Y-YM. Y-YM, FD, KMc, CPW, WPW and CRK evaluated the eligibility of notifications. Y-YM analysed and interpreted the collected data. ASR advised and provided input in the statistical analysis. Y-YM did the literature reviews, drafted and revised all versions of the manuscript. CRK together with contributions from all others (WPW, MJL, CPW, FD, KMc, GAS and ASR) involved in the data interpretation, revised all versions and approved the final version of the manuscript.

  • Funding This national survey was supported by the BPSU/RCPCH Sir Peter Tizard Research Bursary.

  • Competing interests MJL receives research grants from Action Medical Research, DES society, GOSH charity, NIHR, MS Society, SPARKS charity; receives research support grants from the London Clinical Research Network and Evelina Appeal; has received consultation fees from CSL Behring; received travel grants from Merck Serono and awarded educational grants to organise meetings by Novartis, Biogen Idec, Merck Serono and Bayer. GAS has been an investigator in MPSII clinical trials with Shire, has received speaker's honorarium and travel support from BioMarin.

  • Ethics approval The East London & the City Research Ethics Committee (07/Q0603/47), the Patient Information Advisory Group (PIAG/BPSU1-05[FT3]/2007) and the North Wales NHS Research & Development (JJ/jh/RP07/02/02).

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement All authors are willing to share all unpublished data from the study with bona fide researchers. The database can be made available to them through discussion with the corresponding author and the BPSU.

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Linked Articles

  • Atoms
    Martin Ward Platt