Diagnosis and treatment of idiopathic intracranial
hypertension

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

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.

Related collections

Most cited references 33

Record: found
Abstract: found
Article: found

Is Open Access

Idiopathic intracranial hypertension: consensus guidelines on management

Susan Mollan, Brendan Davies, Nick Silver … (2018)

The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH). Methods Between September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements. Results Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist. Conclusions In collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.

0 comments Cited 153 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions.

Keira Markey, Susan Mollan, Rigmor Jensen … (2016)

Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus, and back and neck pain, but the clinical presentation is highly variable. Although few studies have been done to support evidence-based management, several recent advances have the potential to enhance understanding of the causes of the disease and to guide treatment decisions. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway. The incidence of idiopathic intracranial hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients.

0 comments Cited 134 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial.

Michael Wall, Michael McDermott, Karl Kieburtz … (2014)

Acetazolamide is commonly used to treat idiopathic intracranial hypertension (IIH), but there is insufficient information to establish an evidence base for its use. To determine whether acetazolamide is beneficial in improving vision when added to a low-sodium weight reduction diet in patients with IIH and mild visual loss. Multicenter, randomized, double-masked, placebo-controlled study of acetazolamide in 165 participants with IIH and mild visual loss who received a low-sodium weight-reduction diet. Participants were enrolled at 38 academic and private practice sites in North America from March 2010 to November 2012 and followed up for 6 months (last visit in June 2013). All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation (PMD) between -2 dB and -7 dB. The mean age was 29 years and all but 4 participants were women. Low-sodium weight-reduction diet plus the maximally tolerated dosage of acetazolamide (up to 4 g/d) or matching placebo for 6 months. The planned primary outcome variable was the change in PMD from baseline to month 6 in the most affected eye, as measured by Humphrey Field Analyzer. Perimetric mean deviation is a measure of global visual field loss (mean deviation from age-corrected normal values), with a range of 2 to -32 dB; larger negative values indicate greater vision loss. Secondary outcome variables included changes in papilledema grade, quality of life (Visual Function Questionnaire 25 [VFQ-25] and 36-Item Short Form Health Survey), headache disability, and weight at month 6. The mean improvement in PMD was greater with acetazolamide (1.43 dB, from -3.53 dB at baseline to -2.10 dB at month 6; n = 86) than with placebo (0.71 dB, from -3.53 dB to -2.82 dB; n = 79); the difference was 0.71 dB (95% CI, 0 to 1.43 dB; P = .050). Mean improvements in papilledema grade (acetazolamide: -1.31, from 2.76 to 1.45; placebo: -0.61, from 2.76 to 2.15; treatment effect, -0.70; 95% CI, -0.99 to -0.41; P < .001) and vision-related quality of life as measured by the National Eye Institute VFQ-25 (acetazolamide: 8.33, from 82.97 to 91.30; placebo: 1.98, from 82.97 to 84.95; treatment effect, 6.35; 95% CI, 2.22 to 10.47; P = .003) and its 10-item neuro-ophthalmic supplement (acetazolamide: 9.82, from 75.45 to 85.27; placebo: 1.59, from 75.45 to 77.04; treatment effect, 8.23; 95% CI, 3.89 to 12.56; P < .001) were also observed with acetazolamide. Participants assigned to acetazolamide also experienced a reduction in weight (acetazolamide: -7.50 kg, from 107.72 kg to 100.22 kg; placebo: -3.45 kg, from 107.72 kg to 104.27 kg; treatment effect, -4.05 kg, 95% CI, -6.27 to -1.83 kg; P < .001). In patients with IIH and mild visual loss, the use of acetazolamide with a low-sodium weight-reduction diet compared with diet alone resulted in modest improvement in visual field function. The clinical importance of this improvement remains to be determined. clinicaltrials.gov Identifier: NCT01003639.

0 comments Cited 109 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Journal

Journal ID (nlm-ta): Cephalalgia

Journal ID (iso-abbrev): Cephalalgia

Journal ID (publisher-id): CEP

Journal ID (hwp): spcep

Title: Cephalalgia

Publisher: SAGE Publications (Sage UK: London, England )

ISSN (Print): 0333-1024

ISSN (Electronic): 1468-2982

Publication date (Electronic): 25 February 2021

Publication date (Print): April 2021

Volume: 41

Issue: 4 , Special Issue: Secondary Headache

Pages: 472-478

Affiliations

[1 ]Department of Paediatrics, Strabismus and Neuro-ophthalmology, Moorfields Eye Hospital, London, UK

[2 ]Department of Ophthalmology, Royal London Hospital, Barts Health NHS Trust, London, UK

[3 ]Wolfson Centre for Age-Related Diseases, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, London, UK

[4 ]NIHR-Wellcome Trust King’s Clinical Research Facility/SLaM Biomedical Research Centre, King’s College Hospital, London, UK

Author notes

[*]Jan Hoffmann, Wolfson Centre for Age-Related Diseases, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, London SE5 9PJ, UK. Email: jan.hoffmann@ 123456kcl.ac.uk

Author information

Jan Hoffmann https://orcid.org/0000-0002-2103-9081

Article

Publisher ID: 10.1177_0333102421997093

DOI: 10.1177/0333102421997093

PMC ID: 8020303

PubMed ID: 33631966

SO-VID: a4e9b8eb-b387-48f2-8efd-3328a0756c91

License:

This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

History

Date received : 9 September 2020

Date revision received : 1 February 2021

Date accepted : 2 February 2021

Custom metadata

typesetter ts2

ScienceOpen disciplines: Neurology

Keywords: idiopathic intracranial hypertension (iih),headache,pain,papilledema

Data availability:

ScienceOpen disciplines: Neurology

Keywords: idiopathic intracranial hypertension (iih), headache, pain, papilledema

Diagnosis and treatment of idiopathic intracranial hypertension

Read this article at

Abstract

Objective

Discussion

Conclusion

Related collections

Karger: Neurology and Neuroscience

Most cited references 33

Idiopathic intracranial hypertension: consensus guidelines on management

Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions.

Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial.

Author and article information

Journal

Affiliations

Author notes

Author information

Article

History

Categories

Custom metadata

Comments

Comment on this article

Similar content 594

Cited by 6

Most referenced authors 253