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Hoffmann et al. The Journal of Headache and Pain (2018) 19:93 The Journal of Headache
/10.1186/s10194-018-0919-2
and Pain
CONSENSUS ARTICLE Open Access
European headache federation guideline
on idiopathic intracranial hypertension
1* 2 3 4 5
Jan Hoffmann , Susan P Mollan , Koen Paemeleire , Christian Lampl , Rigmor H Jensen
and Alexandra J Sinclair6
Abstract
Background: Idiopathic Intracranial Hypertension (IIH) is characterized by an elevation of intracranial pressure (ICP
no identifiable cause. The aetiology remains largely unknown, however observations made in a number of recent
clinical studies are increasing the understanding of the disease and now provide the basis for evidence-based
treatment strategies.
Methods: The Embase, CDSR, CENTRAL, DARE and MEDLINE databases were searched up to 1st June 2018. We
analyzed randomized controlled trials and systematic reviews that investigate IIH.
Results: Diagnostic uncertainty, headache morbidity and visual loss are among the highest concerns of clinicians
and patients in this disease area. Research in this field is infrequent due to the rarity of the disease and the lack of
understanding of the underlying pathology.
Conclusions: This European Headache Federation consensus paper provides evidence-based recommendations
and practical advice on the investigation and management of IIH.
Objective Headache Society (IHS) [3] (Table 1). The term ‘pseudo-
Idiopathic Intracranial Hypertension (IIH) is character- tumor cerebri
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