Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema

The pseudotumor cerebri syndrome embraces disorders characterised by raised intracranial pressure, where the commonest symptom is headache (90%). Idiopathic intracranial hypertension without papilloedema (IIHWOP) is increasingly recognised as a source of refractory headache symptoms and resultant ne...

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Main Authors: Susan P. Mollan, Yu Jeat Chong, Olivia Grech, Alex J. Sinclair, Benjamin R. Wakerley
Format: Article
Language:English
Published: MDPI AG 2021-05-01
Series:Life
Subjects:
Online Access:https://www.mdpi.com/2075-1729/11/6/472
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author Susan P. Mollan
Yu Jeat Chong
Olivia Grech
Alex J. Sinclair
Benjamin R. Wakerley
author_facet Susan P. Mollan
Yu Jeat Chong
Olivia Grech
Alex J. Sinclair
Benjamin R. Wakerley
author_sort Susan P. Mollan
collection DOAJ
description The pseudotumor cerebri syndrome embraces disorders characterised by raised intracranial pressure, where the commonest symptom is headache (90%). Idiopathic intracranial hypertension without papilloedema (IIHWOP) is increasingly recognised as a source of refractory headache symptoms and resultant neurological disability. Although the majority of patients with IIHWOP are phenotypically similar to those with idiopathic intracranial hypertension (IIH), it remains uncertain as to whether IIHWOP is nosologically distinct from IIH. The incidence, prevalence, and the degree of association with the world-wide obesity epidemic is unknown. Establishing a diagnosis of IIHWOP can be challenging, as often lumbar puncture is not routinely part of the work-up for refractory headaches. There are published diagnostic criteria for IIHWOP; however, some report uncertainty regarding a pathologically acceptable cut off for a raised lumbar puncture opening pressure, which is a key criterion. The literature provides little information to help guide clinicians in managing patients with IIHWOP. Further research is therefore needed to better understand the mechanisms that drive the development of chronic daily headaches and a relationship to intracranial pressure; and indeed, whether such patients would benefit from therapies to lower intracranial pressure. The aim of this narrative review was to perform a detailed search of the scientific literature and provide a summary of historic and current opinion regarding IIHWOP.
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spelling doaj.art-cc709bb05e6e46efb84122ec375684072023-11-21T21:07:30ZengMDPI AGLife2075-17292021-05-0111647210.3390/life11060472Current Perspectives on Idiopathic Intracranial Hypertension without PapilloedemaSusan P. Mollan0Yu Jeat Chong1Olivia Grech2Alex J. Sinclair3Benjamin R. Wakerley4Birmingham Neuro-Ophthalmology Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UKBirmingham Neuro-Ophthalmology Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham B15 2TH, UKMetabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston B15 2TT, UKMetabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston B15 2TT, UKMetabolic Neurology, Institute of Metabolism and Systems Research, University of Birmingham, Edgbaston B15 2TT, UKThe pseudotumor cerebri syndrome embraces disorders characterised by raised intracranial pressure, where the commonest symptom is headache (90%). Idiopathic intracranial hypertension without papilloedema (IIHWOP) is increasingly recognised as a source of refractory headache symptoms and resultant neurological disability. Although the majority of patients with IIHWOP are phenotypically similar to those with idiopathic intracranial hypertension (IIH), it remains uncertain as to whether IIHWOP is nosologically distinct from IIH. The incidence, prevalence, and the degree of association with the world-wide obesity epidemic is unknown. Establishing a diagnosis of IIHWOP can be challenging, as often lumbar puncture is not routinely part of the work-up for refractory headaches. There are published diagnostic criteria for IIHWOP; however, some report uncertainty regarding a pathologically acceptable cut off for a raised lumbar puncture opening pressure, which is a key criterion. The literature provides little information to help guide clinicians in managing patients with IIHWOP. Further research is therefore needed to better understand the mechanisms that drive the development of chronic daily headaches and a relationship to intracranial pressure; and indeed, whether such patients would benefit from therapies to lower intracranial pressure. The aim of this narrative review was to perform a detailed search of the scientific literature and provide a summary of historic and current opinion regarding IIHWOP.https://www.mdpi.com/2075-1729/11/6/472diplopiaheadacheidiopathic intracranial hypertension without papilloedemaintracranial pressurelumbar puncturemigraine
spellingShingle Susan P. Mollan
Yu Jeat Chong
Olivia Grech
Alex J. Sinclair
Benjamin R. Wakerley
Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema
Life
diplopia
headache
idiopathic intracranial hypertension without papilloedema
intracranial pressure
lumbar puncture
migraine
title Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema
title_full Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema
title_fullStr Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema
title_full_unstemmed Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema
title_short Current Perspectives on Idiopathic Intracranial Hypertension without Papilloedema
title_sort current perspectives on idiopathic intracranial hypertension without papilloedema
topic diplopia
headache
idiopathic intracranial hypertension without papilloedema
intracranial pressure
lumbar puncture
migraine
url https://www.mdpi.com/2075-1729/11/6/472
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