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Review
. 2020 Dec;267(12):3776-3784.
doi: 10.1007/s00415-020-10090-4. Epub 2020 Jul 22.

Emerging themes in idiopathic intracranial hypertension

Olivia Grech  1   2 , Susan P Mollan  3 , Benjamin R Wakerley  4 , Zerin Alimajstorovic  1   2 , Gareth G Lavery  1   2 , Alexandra J Sinclair  5   6   7
Affiliations
Review

Emerging themes in idiopathic intracranial hypertension

Olivia Grech et al. J Neurol. 2020 Dec.

Abstract

Purpose: Idiopathic intracranial hypertension (IIH) is a rare disorder characterised by raised intracranial pressure. The underlying pathophysiology is mostly unknown and effective treatment is an unmet clinical need in this disease. This review evaluates key emerging themes regarding disease characteristics, mechanisms contributing to raised intracranial pressure and advances in potential therapeutic targets.

Findings: IIH is becoming more common, with the incidence rising in parallel with the global obesity epidemic. Current medical management remains centred around weight management, which is challenging. Metabolic investigations of patients have identified specific androgen profiles in cerebrospinal fluid (CSF), which suggest an endocrine dysfunction impacting CSF secretion in IIH. Glucagon-like peptide-1 (GLP-1) and 11β-hydroxysteroid dehydrogenase type 1 (11β-HSD1) have been found to play a role in CSF dynamics in IIH and have formed the basis of the first clinical trials looking at new treatments.

Conclusions: Identification of novel molecular targets thought to underlie IIH pathology is now being translated to clinical trials.

Keywords: Glucagon-like peptide-1 (GLP-1); Headache; Idiopathic intracranial hypertension; Intracranial pressure; Obesity; Papilloedema.

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Conflict of interest statement

OG—consultancy work for Invex therapeutics (2020). SPM—Invex therapeutics advisory board (2019); Heidelberg engineering speaker fees (2019). BW—consultancy work for Invex therapeutics (2020). ZA—no conflicts. GL—no conflicts. AJS—Novartis and Allergan Advisory board. Speaker fees Novartis. Invex therapeutics, company director with salary and stock options (2019, 2020).

Figures

Fig. 1
Fig. 1
Optical coherence tomography (OCT) images from a young Caucasian woman who presented with papilloedema. CT head and CT venogram showed no abnormalities. CSF opening pressure was 52 cm CSF, with normal contents. Her weight was 99 kg and body mass index 40.2 kg/m2. Following lifestyle intervention of a calorie-controlled diet and exercise alone, she lost 15 kg (BMI 34.1) putting her disease into complete remission within 8 weeks. a Infrared image of the right optic nerve; b infrared image of the right optic nerve following weight loss; c shows the central cross section, and the amount of reduction in the retinal nerve fibre layer in the right eye over the 8 weeks. d Infrared image of the left optic nerve; e infrared image of the left optic nerve following weight loss; f shows the central cross section and the amount of reduction in the retinal nerve fibre layer in the left eye over the 8 weeks
Fig. 2
Fig. 2
Segmentation error is common in higher grades of papilloedema. a The infrared image of the right eye with a Frisén grade 3 disc swelling, as graded on biomicroscopy. b The blue line, segmenting the retinal nerve fibre layer (RNFL), has been automatically placed in the incorrect area. c the manual resegmentation of the RNFL. d The initial figures for the retinal nerve fibre layer values in each segment. e The resegmented values of the RNFL, and can be compared directly to d, highlighting the difference that might clearly change clinical management when longitudinally following a patient
See this image and copyright information in PMC

Comment in

References

    1. Markey KA, Mollan SP, Jensen RH, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. Lancet Neurol. 2016;15(1):78–91. - PubMed
    1. Mollan SP, Davies B, Silver NC, Shaw S, Mallucci CL, Wakerley BR, Krishnan A, Chavda SV, Ramalingam S, Edwards J, et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018;89(10):1088–1100. - PMC - PubMed
    1. Mollan SP, Aguiar M, Evison F, Frew E, Sinclair AJ. The expanding burden of idiopathic intracranial hypertension. Eye (Lond) 2018;33(3):478–485. - PMC - PubMed
    1. McCluskey G, Doherty-Allan R, McCarron P, Loftus AM, McCarron LV, Mulholland D, McVerry F, McCarron MO. Meta-analysis and systematic review of population-based epidemiological studies in idiopathic intracranial hypertension. Eur J Neurol. 2018;25(10):1218–1227. - PubMed
    1. Wall M, Kupersmith MJ, Kieburtz KD, Corbett JJ, Feldon SE, Friedman DI, Katz DM, Keltner JL, Schron EB, McDermott MP, et al. The idiopathic intracranial hypertension treatment trial: clinical profile at baseline. JAMA Neurol. 2014;71(6):693–701. - PMC - PubMed

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