Background: Cognitive disturbance is not pathognomonic of idiopathic intracranial hypertension (IIH), and therefore is not routinely assessed unless it presents as a major complaint. Consequently, cognition has been slow to gain substantial traction in IIH-related research, despite its notable impact on a subset of patients. We completed a systematic review of the literature examining the neurocognitive profile of patients with IIH. Methods: A PRISMA compliant literature search was conducted in Ovid Medline, PubMed, PsycInfo, Embase, Web of Science, Cochrane, Cinahl, and Scopus databases. The initial query yielded 1376 unique articles. These articles were narrowed to those including empirical analyses of cognitive assessment in adult patients with IIH. Results: A final cohort of nine articles resulted, comprising the findings from 309 patients with IIH and 153 healthy control subjects. Although there was considerable variability in methodology particularly with respect to cognitive assessment, fairly consistent deficits were observed across studies in the domains of processing speed, working memory, sustained and complex attention, set-shifting, and confrontation naming. Body mass index and body weight were not associated with cognitive performance. Pertinent limitations of the literature were identified, most notably failure to report trial-level cognitive testing data, the need for more comprehensive testing batteries with less reliance on screening tools, and not controlling for variables that may impact cognition. Conclusions: A more complete understanding of the cognitive profile in patients with idiopathic intracranial hypertension could lead to the increased -relevance of cognitive screening in disease management, and therefore more appropriate neuropsychological referral, earlier identification of functional limitations, and targeted neurorehabilitation.
Keywords: Idiopathic intracranial hypertension; cognition; neuropsychological testing.