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Review
. 2021 Apr;41(4):472-478.
doi: 10.1177/0333102421997093. Epub 2021 Feb 25.

Diagnosis and treatment of idiopathic intracranial hypertension

Naz Raoof  1   2 , Jan Hoffmann  3   4
Affiliations
Review

Diagnosis and treatment of idiopathic intracranial hypertension

Naz Raoof et al. Cephalalgia. 2021 Apr.

Abstract

Objective: To review and discuss the clinical presentation and treatment of idiopathic intracranial hypertension.

Discussion: Visual alterations and headache are the two main symptoms of idiopathic intracranial hypertension, although additional features including cranial nerve palsies, cognitive deficits, olfactory deficits and tinnitus are not uncommon. The headache associated with idiopathic intracranial hypertension frequently has a migrainous phenotype. The underlying cause of the disorder has not yet been elucidated. Several hypotheses have been postulated but none of them can explain the full clinical picture. Therapeutic options remain limited, focusing mainly on reduction in body weight and the reduction of CSF production with carbonic anhydrase inhibitors.

Conclusion: The accurate diagnosis of idiopathic intracranial hypertension is essential as visual deterioration due to papilledema may be irreversible. Given its phenotypic similarity and frequent overlap with chronic migraine it is essential to consider idiopathic intracranial hypertension in the diagnostic workup of chronic headache; in particular, when considering its increasing prevalence. Understanding in detail the pathophysiological mechanisms behind the associated headache would also allow study of current and future therapeutic options in a structured way.

Keywords: Idiopathic intracranial hypertension (IIH); headache; pain; papilledema.

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Conflict of interest statement

Declaration of conflicting interests: The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: NR does not report a conflict of interest. JH has received honoraria for serving on advisory boards, consulting activities and/or for presentations from Allergan, Autonomic Technologies Inc, Cannovex BV, Chordate Medical AB, Eli Lilly, Hormosan Pharma, Lundbeck, Novartis, Sanofi and Teva. He has received personal fees for medico-legal work as well as from Sage Publishing, Springer Healthcare and Quintessence Publishing. He has received research support from Bristol Myers Squibb. None of these activities and honoraria are related to the submitted work.

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