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  • Review Article
  • Published:

High-pressure headaches: idiopathic intracranial hypertension and its mimics

Nature Reviews Neurology volume 8, pages 700–710 (2012)Cite this article

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Abstract

Idiopathic intracranial hypertension (IIH) is a rare disorder that typically affects obese women of childbearing age, but can also occur in paediatric populations. Patients usually present with diffuse, daily headache and visual disturbances, but either symptom can occur in isolation. Patients with IIH often have papilloedema; however, IIH without papilloedema is fairly common in patients with chronic daily headache. The pathogenesis of IIH is unknown; the high incidence of comorbid bilateral transverse sinus stenosis (BTSS) in patients with IIH suggests that the two conditions are linked, although no direct causal relationship has been established. Cerebrospinal fluid (CSF) pressure monitoring or lumbar puncture—which provides immediate symptomatic relief—are important in making a diagnosis of IIH. Current treatments for IIH include weight reduction, medical treatment, CSF diversion surgery, optic nerve sheath fenestration and, potentially, endovascular stenting (in patients with BTSS). Prevention of visual loss (which can be substantial) is the main goal of treatment. Residual headache and IIH recurrence are not uncommon after treatment, and regular follow-up is, therefore, warranted even in patients who achieve remission. This Review provides an update of current knowledge of the aetiology, pathophysiology and treatment of IIH.

Key Points

  • Idiopathic intracranial hypertension (IIH) is a rare headache disorder that predominantly affects obese women of childbearing age

  • Diagnosis of IIH requires exclusion of metabolic, endocrine and drug-related aetiologies; magnetic resonance venography is crucial to identify venous thrombosis and bilateral transverse sinus stenosis

  • The most common symptoms of IIH are headache, papilloedema and pulsatile tinnitus; however, patients might not present with all three symptoms

  • In patients with suspected IIH, symptomatic relief resulting from a decrease in CSF pressure (achieved by lumbar puncture) confirms the diagnosis

  • Current treatment options for IIH include lumbar puncture, diversion surgery, optic nerve sheath fenestration, and weight reduction (in obese individuals)

  • Visual loss is likely in patients with IIH who are male, of African ancestry, or have systemic hypertension, anaemia or obesity—close follow-up is recommended for these patient populations

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Figure 1: Magnetic resonance cerebral venography findings in a 32-year-old woman with idiopathic intracranial hypertension.
Figure 2: A possible link between cerebral venous stenosis and increased ICP.

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Acknowledgements

The authors' research work is supported by grants awarded to S.-J. Wang from the Taiwan National Science Council (100-2314-B-010-019-MY2, 100-2314-B-010-018-MY3) and Taipei Veterans General Hospital (VGHUST101-G7-1-1, V101C-106, V101E7-003). The authors also acknowledge National Science Council support for the Center for Dynamic Biomarkers and Translational Medicine, National Central University, Taiwan (NSC 100-2911-I-008-001), Brain Research Center, National Yang-Ming University and a grant from the Ministry of Education, Aim for the Top University Plan.

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  1. Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, No. 201, Sec. 2, Shipai Road, Taipei, 11217, Taiwan

    Kuan-Po Peng, Jong-Ling Fuh & Shuu-Jiun Wang

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K.-P. Peng researched the data for the article. K.-P. Peng, J.-L. Fuh and S.-J. Wang contributed equally to discussions of the content. K.-P. Peng wrote the article, and K.-P. Peng, J.-L. Fuh and S.-J. Wang reviewed and/or edited the manuscript before submission.

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Correspondence to Shuu-Jiun Wang.

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Peng, KP., Fuh, JL. & Wang, SJ. High-pressure headaches: idiopathic intracranial hypertension and its mimics. Nat Rev Neurol 8, 700–710 (2012). https://doi.org/10.1038/nrneurol.2012.223

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