John Glenn Burkett1, Jessica Ailani2. 1. Department of Neurology, Medstar Georgetown University Hospital, 3800 Reservoir Road NW, 7-PHC, Washington, DC, 20007, USA. John.G.Burkett@gunet.georgetown.edu. 2. Department of Neurology, Medstar Georgetown University Hospital, 3800 Reservoir Road NW, 7-PHC, Washington, DC, 20007, USA.
Abstract
PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH), pseudotumor cerebri syndrome (PTCS), and benign intracranial hypertension are all terms that have been used for a neurologic syndrome consisting of elevated intracranial pressure (ICP), headache and vision loss without mass lesion or underlying infection or malignancy. In this review article, categorization, diagnostic criteria, symptom management strategies, and disease treatment options for pseudotumor cerebri syndrome will be discussed. RECENT FINDINGS: The Idiopathic Intracranial Hypertension Treatment Trial has now proven that acetazolamide should be the first line therapy in primary PTCS, but other treatment options exist in patients who cannot tolerate acetazolamide or in selected cases, which requires surgical intervention for PTCS which acutely threatens vision. Headache has also been shown to require focused treatment beyond therapies that lower ICP, specifically targeting coexistent primary headache disorders and medication overuse. Advances in treatment and diagnostic modalities have improved understanding of PTCS types and their treatment. The pathophysiology of primary PTCS, however, remains incompletely understood, but continued evaluation of cerebrospinal fluid flow dynamics, aquaporins, hormones, natriuretic peptides, and the link with female gender and obesity may lead to future answers.
PURPOSE OF REVIEW: Idiopathic intracranial hypertension (IIH), pseudotumor cerebri syndrome (PTCS), and benign intracranial hypertension are all terms that have been used for a neurologic syndrome consisting of elevated intracranial pressure (ICP), headache and vision loss without mass lesion or underlying infection or malignancy. In this review article, categorization, diagnostic criteria, symptom management strategies, and disease treatment options for pseudotumor cerebri syndrome will be discussed. RECENT FINDINGS: The Idiopathic Intracranial Hypertension Treatment Trial has now proven that acetazolamide should be the first line therapy in primary PTCS, but other treatment options exist in patients who cannot tolerate acetazolamide or in selected cases, which requires surgical intervention for PTCS which acutely threatens vision. Headache has also been shown to require focused treatment beyond therapies that lower ICP, specifically targeting coexistent primary headache disorders and medication overuse. Advances in treatment and diagnostic modalities have improved understanding of PTCS types and their treatment. The pathophysiology of primary PTCS, however, remains incompletely understood, but continued evaluation of cerebrospinal fluid flow dynamics, aquaporins, hormones, natriuretic peptides, and the link with female gender and obesity may lead to future answers.
Authors: Michael Wall; Mark J Kupersmith; Karl D Kieburtz; James J Corbett; Steven E Feldon; Deborah I Friedman; David M Katz; John L Keltner; Eleanor B Schron; Michael P McDermott Journal: JAMA Neurol Date: 2014-06 Impact factor: 18.302
Authors: A Sundholm; S Burkill; O Sveinsson; F Piehl; S Bahmanyar; A I M Nilsson Remahl Journal: Acta Neurol Scand Date: 2017-02-28 Impact factor: 3.209
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