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      Idiopathic intracranial hypertension: consensus guidelines on management

      review-article
      1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 4 , 10 , 10 , 11 , 12 , 13 , 13 , 2 , 1 , 12 , 14 , 15 , 16 , 1 , 2 , 12 , 17
      (Collab), (Collab), (Collab), (Collab), (Collab), (Collab)
      Journal of Neurology, Neurosurgery, and Psychiatry
      BMJ Publishing Group
      headache, benign intracran hyp, clinical neurology, neuroophthalmology, neurosurgery

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          Abstract

          The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH).

          Methods

          Between September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements.

          Results

          Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist.

          Conclusions

          In collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.

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          Most cited references65

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          Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children.

          The pseudotumor cerebri syndrome (PTCS) may be primary (idiopathic intracranial hypertension) or arise from an identifiable secondary cause. Characterization of typical neuroimaging abnormalities, clarification of normal opening pressure in children, and features distinguishing the syndrome of intracranial hypertension without papilledema from intracranial hypertension with papilledema have furthered our understanding of this disorder. We propose updated diagnostic criteria for PTCS to incorporate advances and insights into the disorder realized over the past 10 years.
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            Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions.

            Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus, and back and neck pain, but the clinical presentation is highly variable. Although few studies have been done to support evidence-based management, several recent advances have the potential to enhance understanding of the causes of the disease and to guide treatment decisions. Investigators of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway. The incidence of idiopathic intracranial hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients.
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              Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis.

              To determine the prevalence and nature of sinovenous obstruction in idiopathic intracranial hypertension (IIH) using auto-triggered elliptic-centric-ordered three-dimensional gadolinium-enhanced MR venography (ATECO MRV). In a prospective controlled study, 29 patients with established IIH as well as 59 control patients underwent ATECO MRV. In a randomized blinded fashion, three readers evaluated the images. Using a novel scoring system, each reader graded the degree of stenosis seen in the transverse and sigmoid sinuses of each patient. There was excellent agreement across the three readers for application of the grading system. Substantial bilateral sinovenous stenoses were seen in 27 of 29 patients with IIH and in only 4 of 59 control patients. Using ATECO MRV and a novel grading system for quantifying sinovenous stenoses, the authors can identify IIH patients with sensitivity and specificity of 93%.
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                Author and article information

                Journal
                J Neurol Neurosurg Psychiatry
                J. Neurol. Neurosurg. Psychiatry
                jnnp
                jnnp
                Journal of Neurology, Neurosurgery, and Psychiatry
                BMJ Publishing Group (BMA House, Tavistock Square, London, WC1H 9JR )
                0022-3050
                1468-330X
                October 2018
                14 June 2018
                : 89
                : 10
                : 1088-1100
                Affiliations
                [1 ] departmentMetabolic Neurology, Institute of Metabolism and Systems Research , University of Birmingham , Birmingham, UK
                [2 ] departmentBirmingham Neuro-Ophthalmology , Queen Elizabeth Hospital , Birmingham, UK
                [3 ] departmentDepartment of Neurology , University Hospital North Midlands NHS Trust , Stoke-on-Trent, UK
                [4 ] departmentDepartment of Neurology , The Walton Centre NHS Foundation Trust , Liverpool, UK
                [5 ] departmentDepartment of Neurosurgery , University Hospital North Midlands NHS Trust, Royal Stoke University Hospital , Stoke-on-Trent, UK
                [6 ] departmentDepartment of Neurosurgery , The Walton Centre NHS Foundation Trust , Liverpool, UK
                [7 ] departmentDepartment of Paediatric Neurosurgery , Alder Hey Children’s NHS Foundation Trust , Liverpool, UK
                [8 ] departmentDepartment of Neurology , Gloucestershire Hospitals NHS Foundation Trust , Cheltenham, UK
                [9 ] departmentNuffield Department of Clinical Neurosciences , John Radcliffe Hospital , Oxford, UK
                [10 ] departmentDepartment of Neuroradiology , University Hospitals Birmingham, Queen Elizabeth Hospital , Birmingham, UK
                [11 ] departmentDepartment of Neurology , Sandwell and West Birmingham NHS Trust , Birmingham, UK
                [12 ] departmentDepartment of Neurology , University Hospitals Birmingham, Queen Elizabeth Hospital , Birmingham, UK
                [13 ] IIH-UK charity , Tyne & Wear, UK
                [14 ] departmentDepartments of Ophthalmology and Neurology , Moran Eye Center, University of Utah , Salt Lake City, Utah, USA
                [15 ] departmentNeuro-ophthalmology Services , Children’s Hospital of Philadelphia and Hospital of the University of Pennsylvania , Philadelphia, Pennsylvania, USA
                [16 ] departmentDanish Headache Center, Department of Neurology , Rigshospitalet-Glostrup, University of Copenhagen , Copenhagen, Denmark
                [17 ] departmentCentre for Endocrinology, Diabetes and Metabolism , Birmingham Health Partners , Birmingham, UK
                Author notes
                [Correspondence to ] Dr Alexandra J Sinclair, Metabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Birmingham B15 2TT, UK; a.b.sinclair@ 123456bham.ac.uk
                Author information
                http://orcid.org/0000-0002-6314-4437
                http://orcid.org/0000-0002-6314-4437
                Article
                jnnp-2017-317440
                10.1136/jnnp-2017-317440
                6166610
                29903905
                f673913c-5072-43cb-a182-b37a01c60eb0
                © Author(s) (or their employer(s)) 2018. Re-use permitted under CC BY. Published by BMJ.

                This is an open access article distributed in accordance with the Creative Commons Attribution 4.0 Unported (CC BY 4.0) license, which permits others to copy, redistribute, remix, transform and build upon this work for any purpose, provided the original work is properly cited, a link to the licence is given, and indication of whether changes were made. See: https://creativecommons.org/licenses/by/4.0/.

                History
                : 12 November 2017
                : 06 February 2018
                : 07 February 2018
                Categories
                General Neurology
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                1507
                Review
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                Surgery
                headache,benign intracran hyp,clinical neurology,neuroophthalmology,neurosurgery
                Surgery
                headache, benign intracran hyp, clinical neurology, neuroophthalmology, neurosurgery

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