Update on the pathophysiology and management of idiopathic intracranial hypertension
J Neurol Neurosurg Psychiatry doi:10.1136/jnnp-2011-302029
Author Affiliation
Department of Ophthalmology, Emory University, Atlanta, Georgia, USA
Department of Neurology, Emory University, Atlanta, Georgia, USA
Department of Neurological Surgery, Emory University, Atlanta, Georgia, USA
Contributors All three authors contributed to the review of the literature, and the preparation of the manuscript and figures.
Received 13 December 2011
Revised 20 January 2012
Accepted 25 January 2012
Published Online First 15 March 2012
Abstract
Idiopathic intracranial hypertension is a disease of unknown aetiology, typically affecting young obese women, producing a syndrome of increased intracranial pressure without identifiable cause. Despite a large number of hypotheses and publications over the past decade, the aetiology is still unknown. 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. There continues to be no evidence based consensus or formal guidelines regarding management and treatment of the disease. Treatment studies show that the diagnostic lumbar puncture is a valuable intervention beyond its diagnostic importance, and that weight management is critical. However, many questions remain regarding the efficacy of acetazolamide, CSF shunting procedures and cerebral transverse venous sinus stenting.
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