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Idiopathic intracranial hypertension: ongoing clinical challenges and future prospects

Authors Julayanont P, Karukote A, Ruthirago D, Panikkath D, Panikkath R

Received 3 August 2015

Accepted for publication 30 October 2015

Published 19 February 2016 Volume 2016:9 Pages 87—99

DOI https://doi.org/10.2147/JPR.S60633

Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Dr Michael Schatman


Parunyou Julayanont,1 Amputch Karukote,2 Doungporn Ruthirago,1 Deepa Panikkath,3 Ragesh Panikkath3

1Department of Neurology, Texas Tech University Health Science Center, Lubbock, TX, USA; 2Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 3Department of Internal Medicine, Texas Tech University Health Science Center, Lubbock, TX, USA

Abstract: Idiopathic intracranial hypertension (IIH) is an uncommon disorder characterized by increased intracranial pressure without radiological or laboratory evidence of intracranial pathology except empty sella turcica, optic nerve sheath with filled out cerebrospinal fluid spaces, and smooth-walled nonflow-related venous sinus stenosis or collapse. This condition typically affects obese women. The incidence of IIH is increasing with the rising prevalence of obesity. Persistent headache is the most common symptom. Visual impairment is a serious complication that may not be recognized by the patients. This paper reviews clinical manifestations, diagnostic challenges, and current treatments of IIH in adults. Various imaging modalities have been studied on their validity for detection of IIH and papilledema. This review also includes new studies on medical, surgical, and interventional management of this condition. Acetazolamide and topiramate are the only two medications that have been studied in randomized controlled trials about their efficacy in treatment of IIH. In patients who have severe visual impairment or progressive visual deterioration despite medical management, surgical or interventional treatment may be considered. The efficacy and complications of cerebrospinal fluid diversion, optic nerve sheath fenestration, and endovascular venous stenting reported in the last 3 decades have been summarized in this review. Finally, the prospective aspects of biomarkers and treatments are proposed for future research.

Keywords: acetazolamide, cerebrospinal fluid shunts, endovascular stenting, optic nerve sheath fenestration, pseudotumor cerebri

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