Different Characteristics of Pre-Pubertal and Post-Pubertal Idiopathic Intracranial Hypertension: A Narrative Review

ABSTRACT

Idiopathic intracranial hypertension (IIH) affects both children and adults. There are currently no clinical trials in IIH for those who are adolescents or children. The aims of this narrative review were to characterise the differences between pre- and post-pubertal IIH and to highlight the need to be more inclusive in clinical trial planning and recruitment. A detailed search of the scientific literature was performed using the PubMed database, from inception until 30 May 2022 using keywords. This included English language papers only. The abstracts and full texts were reviewed by two independent assessors. The literature revealed that the pre-pubertal group had a more variable presentation. The presenting features in the post-pubertal paediatric group were more akin to adults with headache as the dominant feature. They were also more likely to be female and have an increased body mass index. A clear limitation of the literature was that a number of paediatric studies had variable inclusion criteria, including secondary causes of raised intracranial pressure. Pre-pubertal children do not display the same predilection towards the female sex and obesity as post-pubertal children, who have a similar phenotype to the adult cohort. Inclusion of adolescents in clinical trials should be considered given the similar phenotype to adults. There is a lack of consistency in the definition of puberty, making the IIH literature difficult to compare. Inclusion of secondary causes of raised intracranial pressure has the potential to confound the accuracy of analysis and interpretation of the results.

KEYWORDS:

• Adolescent
• pseudotumour cerebri
• paediatrics
• intracranial pressure
• papilloedema
• acetazolamide

Acknowledgments

We wish to acknowledge that the infographic figures were created with Biorender.com.

Disclosure statement

SPM reports consultancy fees (Invex Therapeutics); advisory board fees (Invex therapeutics; Gensight) and speaker fees (Heidelberg engineering; Chugai-Roche Ltd; Allergan; Santen; Teva UK; Chiesi; and Santhera). AJS reports personal fees from Invex therapeutics during the conduct of the study as well as share options and shareholdings, speaker fees (Novartis; Allergan; Teva UK) and consulting fees (Allergan; Chiesi; Novartis; Lundbeck). The other authors report no conflict of interest. Authors declare no other financial relationships with any organisations that might have an interest in the submitted work; and no other relationships or activities that could appear to have influenced the submitted work.

Additional information

Funding

Sinclair is supported by a Sir Jules Thorn Award for Biomedical Research and the National Institute of Health Research.