Idiopathic intracranial hypertension

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Last reviewed: 20 Aug 2025

Last updated: 04 Feb 2022

Idiopathic intracranial hypertension (IIH) is a disorder of increased intracranial pressure in an alert and oriented patient. The most popular hypothesis is that IIH is a syndrome of reduced cerebrospinal fluid absorption.

Clinical features include headaches, pulse-synchronous tinnitus, transient visual obscurations, visual loss, neck and back pain, and diplopia.

Signs include papilledema, sixth nerve paresis, and disturbances in sensory visual function. Visual field loss is ubiquitous, and the prototype pattern for early loss is enlargement of the blind spot and inferonasal loss. Diagnostic criteria are the modified Dandy criteria.

In all patients with IIH, treatment consists of eliminating causal factors, such as drugs and other conditions known to cause increased intracranial pressure, and instituting a low-sodium weight-reduction diet plus acetazolamide when indicated. Therapy can be given to reverse and prevent loss of vision.

Definição

IIH, also known as pseudotumor cerebri, is a disorder of increased intracranial pressure that occurs mainly in overweight women of childbearing years, often in the setting of weight gain.[1] Its cause is unknown. It is characterized by increased intracranial pressure and its associated signs and symptoms in an alert and oriented patient, but without localizing neurologic ﬁndings. There is no evidence of deformity or obstruction of the ventricular system, and neurodiagnostic studies are normal except for increased cerebrospinal fluid pressure and the related neuroimaging signs. Furthermore, no secondary cause of intracranial hypertension is apparent. IIH can either be self-limited or have a life-long chronic course.[1][2]

História e exame físico

Principais fatores diagnósticos

visual field loss

Detalhes completos

Outros fatores diagnósticos

headache
transient visual obscurations
pulse-synchronous tinnitus
photophobia
retrobulbar pain
optical disk swelling
decreased visual acuity
ocular motility disturbances
relative afferent pupillary defect

Detalhes completos

Fatores de risco

female sex
obesity and weight gain
certain medication use
associated causal diseases
sleep apnea
family history

Detalhes completos

Investigações diagnósticas

Primeiras investigações a serem solicitadas

visual field testing (perimetry)
dilated fundoscopy
visual acuity
MRI of brain with or without contrast
lumbar puncture at spinal L3/L4

Detalhes completos

Investigações a serem consideradas

magnetic resonance venogram of head
optical coherence tomography

Detalhes completos

Algoritmo de tratamento

AGUDA

all patients

Colaboradores

Autores

Michael Wall, MD

Professor

Department of Neurology and Department of Ophthalmology & Visual Sciences

University of Iowa Hospitals & Clinics and Iowa City VA Health Care System

Iowa City

Declarações

MW is an author of a number of references cited in this topic.

Mansoor Mughal, MD

Retina Fellow

Rutgers University

Robert Wood Johnson University Hospital

New Brunswick

Declarações

MM declares that he has no competing interests.

Revisores

Paul W. Brazis, MD

Consultant in Neurology and Neuro-Ophthalmology

Mayo Clinic Florida

Jacksonville

Declarações

PWB declares that he has no competing interests.

Tim D. Matthews, MBBS

Consultant Neuro-ophthalmologist

Birmingham Neuro-ophthalmology Unit

University Hospital Birmingham

Birmingham

Declarações

TDM declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Wall M. Idiopathic intracranial hypertension. Neurol Clin. 2010 Aug;28(3):593-617.Texto completo Resumo

Johnson LN, Krohel GB, Madsen RW, et al. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. 1998 Dec;105(12):2313-7. Resumo

Hayreh SS. Pathogenesis of oedema of the optic disc (papilloedema): a preliminary report. Br J Ophthalmol. 1964 Oct;48:522-43.Texto completo Resumo

Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol. 1985 Mar;5(1):55-6. Resumo

Frisen L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry. 1982 Jan;45(1):13-8.Texto completo Resumo

NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee; Wall M, McDermott MP, Kieburtz KD, et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014 Apr 23-30;311(16):1641-51.Texto completo Resumo

Hoffmann J, Mollan SP, Paemeleire K, et al. European Headache Federation guideline on idiopathic intracranial hypertension. J Headache Pain. 2018 Oct 8;19(1):93.Texto completo Resumo

Sinclair AJ, Burdon MA, Nightingale PG, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: prospective cohort study. BMJ. 2010 Jul 7;341:c2701.Texto completo Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais
- Intracranial structural anomalies
Mais Diagnósticos diferenciais
Diretrizes
- European Headache Federation guideline on idiopathic intracranial hypertension
Mais Diretrizes
Folhetos informativos para os pacientes
Obesity - drugs and surgery
Obesity - diet and exercise
Mais Folhetos informativos para os pacientes
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Idiopathic intracranial hypertension

Summary

Definição

História e exame físico

Principais fatores diagnósticos

Outros fatores diagnósticos

Fatores de risco

Investigações diagnósticas

Primeiras investigações a serem solicitadas

Investigações a serem consideradas

Algoritmo de tratamento

all patients

Colaboradores

Autores

Michael Wall, MD

Declarações

Mansoor Mughal, MD

Declarações

Revisores

Paul W. Brazis, MD

Declarações

Tim D. Matthews, MBBS

Declarações

Peer reviewer acknowledgements

Disclosures

Referências

Principais artigos

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.

Diagnósticos diferenciais

Diretrizes

Folhetos informativos para os pacientes

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se ou assinar para acessar todo o BMJ Best Practice

Conectar-se para acessar todo o BMJ Best Practice