Adult idiopathic intracranial hypertension without papilledema: systematic review of literature and future perspectives
Labella Alvarez, Fernando Berman, Gabriele Lowe, Michael Kulniwatcharoen, Pichaya Mollan, Susan P
Labella Alvarez, Fernando
Berman, Gabriele
Lowe, Michael
Kulniwatcharoen, Pichaya
Mollan, Susan P
Abstract
Background: Idiopathic intracranial hypertension without papilledema (IIHWOP) remains poorly understood. This review summarizes the diagnostic challenges and potential management of adults with IIHWOP.
Evidence acquisition: A detailed search of the scientific literature, combining MeSH and free-text terms, included all English-language papers on PubMed, from inception to June 8, 2025. In this review, we used the term IIHWOP to describe patients without evidence of active or previous papilledema.
Results: The diagnosis of IIHWOP is based on elevated lumbar puncture opening pressure accompanied by either sixth cranial nerve palsy or neuroimaging features of intracranial hypertension, which may lack specificity. Clinical presentation frequently mimics primary headache disorders, and lumbar puncture remains an invasive procedure without clear management implications in IIHWOP, as there are no high-quality studies evaluating medical or surgical therapies.
Conclusions: Recommendations for investigation and clinical care remain largely inferred from idiopathic intracranial hypertension. Given the absence of evidence for risk of vision loss, invasive procedures should be avoided, and management should focus on weight loss and optimized headache management.
MIDER Authors
Affiliations
University Hospital Zurich; Córdoba University; University Hospitals Birmingham NHS Foundation Trust; University of Birmingham; NHS Greater Glasgow and Clyde; Chiang Mai University Hospital; Queen's University Kingston
Date
2026-06-01
Type
Article
Collections
Citation
Labella Alvarez F, Berman G, Lowe M, Kulniwatcharoen P, Mollan SP. Adult Idiopathic Intracranial Hypertension Without Papilledema: Systematic Review of Literature and Future Perspectives. J Neuroophthalmol. 2026 Jun 1;46(2):149-164. doi: 10.1097/WNO.0000000000002473.