Update on Idiopathic Intracranial Hypertension

Abstract

To provide an update on various features of idiopathic intracranial hypertension. Perspective. Selected articles on the epidemiology, clinical and imaging features, natural history, pathophysiology, and treatment of idiopathic intracranial hypertension were reviewed and interpreted in the context of the authors' clinical and research experience. Idiopathic intracranial hypertension primarily is a disease of obese women of childbearing age, but it can affect patients of any weight, sex, and age. Although a relatively rare disorder, idiopathic intracranial hypertension's associated costs in the United States entail hundreds of millions of dollars. Even after treatment, headaches frequently are persistent and may require the continued involvement of a neurologist. Quality-of-life reductions and depression are common among idiopathic intracranial hypertension patients. However, visual dysfunction, especially visual field abnormalities, represents the major morbidity of this disorder, and serial automated perimetry remains the primary method of patient monitoring. Patients who are men, black, very obese, or anemic are at higher risk of visual loss. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding the pathophysiology of idiopathic intracranial hypertension. Treatment studies show that lumbar puncture is a valuable treatment (in addition to its crucial diagnostic role), and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, cerebrospinal fluid diversion procedures, and cerebral venous stenting. Many questions remain unanswered about idiopathic intracranial hypertension. Ongoing studies, especially an ongoing National Institutes of Health-funded clinical trial of acetazolamide, should provide more insight into this important, yet poorly understood, syndrome of isolated intracranial hypertension.