Adult Idiopathic Intracranial Hypertension Without Papilledema: Systematic Review of Literature and Future Perspectives
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Published on: February 8, 2022
Fernando Labella Alvarez1, Gabriele Berman, Michael Lowe
1Department of Neurology and Ophthalmology (FLA), University Hospital Zurich, Zurich, Switzerland; Department of Morphological and Socio-Sanitary Sciences (FLA), Córdoba University School of Medicine and Nursing, Córdoba, Andalusia, Spain; Birmingham Neuro-Ophthalmology (GB, ML), University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom; Metabolism and Systems Science (GB, SPM), School of Medical Sciences, University of Birmingham, Birmingham, United Kingdom; Department of Neurology (ML), Institute of Neurological Sciences, Queen Elizabeth University Hospital, Glasgow, United Kingdom; Department of Ophthalmology (PK), Chiang Mai University Hospital, Chiang Mai, Thailand; and Ophthalmology Department (SPM), Queen's University, Kingston, Canada.
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.
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