Diagnosis and treatment of idiopathic intracranial hypertension

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


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.

Publication types

  • Review

MeSH terms

  • Acetazolamide / therapeutic use
  • Analgesics / therapeutic use
  • Headache / diagnosis
  • Headache / etiology*
  • Headache / therapy
  • Headache Disorders
  • Humans
  • Intracranial Hypertension
  • Migraine Disorders
  • Papilledema / diagnosis
  • Papilledema / etiology*
  • Pseudotumor Cerebri / complications*
  • Pseudotumor Cerebri / diagnosis
  • Pseudotumor Cerebri / therapy*
  • Vision Disorders / complications*
  • Vision Disorders / therapy
  • Weight Reduction Programs


  • Analgesics
  • Acetazolamide