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Review
. 2018 Oct;89(10):1088-1100.
doi: 10.1136/jnnp-2017-317440. Epub 2018 Jun 14.

Idiopathic intracranial hypertension: consensus guidelines on management

Susan P Mollan  1   2 , Brendan Davies  3 , Nick C Silver  4 , Simon Shaw  5 , Conor L Mallucci  6   7 , Benjamin R Wakerley  8   9 , Anita Krishnan  4 , Swarupsinh V Chavda  10 , Satheesh Ramalingam  10 , Julie Edwards  11   12 , Krystal Hemmings  13 , Michelle Williamson  13 , Michael A Burdon  2 , Ghaniah Hassan-Smith  1   12 , Kathleen Digre  14 , Grant T Liu  15 , Rigmor Højland Jensen  16 , Alexandra J Sinclair  1   2   12   17
Affiliations
Review

Idiopathic intracranial hypertension: consensus guidelines on management

Susan P Mollan et al. J Neurol Neurosurg Psychiatry. 2018 Oct.

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.

Keywords: benign intracran hyp; clinical neurology; headache; neuroophthalmology; neurosurgery.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Consensus in diagnosing IIH. (A) Frequency of IIH symptoms reported, adapted from Markey et al. (B) IIH diagnostic criteria, adapted from Friedman et al. (C) IIHWOP diagnostic criteria, adapted from Friedman et al. (D) Headache attributed to IIH, as described by the International Classification of Headache Disorders, 3rd edition (beta version) (ICHD-3 beta). (E) Line figure detailing the consensus of the interpretation of LP opening pressure. Uncertainty: it needs to be recognised that this is a single LP OP measurement; and after raised ICP what is then a normal ICP for this population on repeat LP readings is unknown. CSF, cerebrospinal fluid; IIH, idiopathic intracranial hypertension; LP, lumboperitoneal.
Figure 2
Figure 2
A flow diagram of investigation of papilloedema. BMI, body mass index; IIH, idiopathic intracranial hypertension.
Figure 3
Figure 3
Management flow chart of diagnosed IIH. BMI, body mass index; CSF, cerebrospinal fluid; IIH, idiopathic intracranial hypertension.
Figure 4
Figure 4
Flow chart of acute exacerbation of headache in IIH with known CSF shunt in situ. CSF, cerebrospinal fluid; IIH, idiopathic intracranial hypertension.
See this image and copyright information in PMC

References

    1. Friesner D, Rosenman R, Lobb BM, et al. . Idiopathic intracranial hypertension in the USA: the role of obesity in establishing prevalence and healthcare costs. Obes Rev 2011;12:e372–80. 10.1111/j.1467-789X.2010.00799.x - DOI - PubMed
    1. Kalyvas AV, Hughes M, Koutsarnakis C, et al. . Efficacy, complications and cost of surgical interventions for idiopathic intracranial hypertension: a systematic review of the literature. Acta Neurochir 2017;159:33–49. 10.1007/s00701-016-3010-2 - DOI - PubMed
    1. Markey KA, Mollan SP, Jensen RH, et al. . Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol 2016;15:78–91. 10.1016/S1474-4422(15)00298-7 - DOI - PubMed
    1. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81:1159–65. 10.1212/WNL.0b013e3182a55f17 - DOI - PubMed
    1. Kilgore KP, Lee MS, Leavitt JA, et al. . Re-evaluating the incidence of idiopathic intracranial hypertension in an era of increasing obesity. Ophthalmology 2017;124:697–700. 10.1016/j.ophtha.2017.01.006 - DOI - PMC - PubMed

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