Idiopathic intracranial hypertension - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

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.

Bilateral disk edema

From the personal collection of Dr M. Wall; used with permission

Quick Reference

History & Exam
- Key Factors
- Other Factors
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Diagnostics Tests
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Treatment Options
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Bilateral disk edema
Magnetic resonance image (MRI) of empty sella on sagittal view
Bilateral disk swelling settled
Bilateral optic atrophy
Frisén stage I
Frisén stage II
Frisén stage III
Frisén stage IV
Frisén stage V
MRI of empty sella on sagittal view

Citations

Key Articles

Wall M. Idiopathic intracranial hypertension. Neurol Clin. 2010 Aug;28(3):593-617.[Abstract][Full Text]
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.[Abstract]
Hayreh SS. Pathogenesis of oedema of the optic disc (papilloedema): a preliminary report. Br J Ophthalmol. 1964 Oct;48:522-43.[Abstract][Full Text]
Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol. 1985 Mar;5(1):55-6.[Abstract]
Frisen L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry. 1982 Jan;45(1):13-8.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]

Referenced Articles

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36. Smith JL. Whence pseudotumor cerebri? J Clin Neuroophthalmol. 1985 Mar;5(1):55-6.[Abstract]
37. Frisen L. Swelling of the optic nerve head: a staging scheme. J Neurol Neurosurg Psychiatry. 1982 Jan;45(1):13-8.[Abstract][Full Text]
38. Wall M, George D. Idiopathic intracranial hypertension: a prospective study of 50 patients. Brain. 1991 Feb;114 (pt 1A):155-80.[Abstract]
39. Friedman DI, Quiros PA, Subramanian PS, et al. Headache in idiopathic intracranial hypertension: Findings from the Idiopathic Intracranial Hypertension Treatment Trial. Headache. 2017 Sep;57(8):1195-205.[Abstract]
40. Wall M. The headache proﬁle of idiopathic intracranial hypertension. Cephalalgia. 1990 Dec;10(6):331-5.[Abstract]
41. Sadun AA, Currie JN, Lessell S. Transient visual obscurations with elevated optic discs. Ann Neurol. 1984 Oct;16(4):489-94.[Abstract]
42. Wall M, Falardeau J, Fletcher WA, et al. Risk factors for poor visual outcome in patients with idiopathic intracranial hypertension. Neurology. 2015 Sep 1;85(9):799-805.[Abstract]
43. Meador KJ, Swift TR. Tinnitus from intracranial hypertension. Neurology. 1984 Sep;34(9):1258-61.[Abstract]
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