European headache federation guideline on idiopathic intracranial hypertension

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.

Fig. 1

Diagnostic criteria for IIH (Friedman criteria). Diagnostic criteria for IIH and IIH without papilloedema. Infogram demonstrating the “grey zone” in which LP pressure is normal in some individuals but can indicate pathologically raised ICP in some. Measurements in the grey zone need to be interpreted with caution and patients must fit the other criteria for IIH for a diagnosis to be confirmed

Fig. 2

Typical visual field defects in IIH. Common visual field defects seen in IIH with the Humphrey visual field analyser grey scale. a, Left eye with a slightly enlarged blind spot; b, right eye with slightly enlarged blind spot; c, Left eye obvious enlarged blind spot; d, right eye with enlarged blind spot and paracentral scotoma; e, left eye with enlarged blind spot and prominent inferior nasal step; f, Left eye with enlarged blind spot, dense superior and inferior arcuate scotomas

Fig. 3

Wide-field imaging using. Wide-field imaging with the Optos™ through an undilated pupil in a, normal patient and b, a patient with IIH. a, normal fundus with blue high magnification box to inspect the optic nerve. Peripapillary atrophy 360^o around the disc which is normal. Note the lashes seen inferiorly as artefact on image. b, right optic nerve which has grade 2 Frisen swelling where there is elevation of the optic disc margin 360^o, loss of the clear optic disc margin as seen in a. c, the high magnification tool allows excellent visualisation of the swelling without degradation of the image

Fig. 4

Optical coherence tomography highlighting improvement of papilloedema. OCT is useful for monitoring of changes in papilloedema. a, Right eye infrared (IR) image of a swollen optic nerve. Note the Paton’s lines (circumferential lines) between 9 o’clock and 11 o’clock. b, Right eye IR image the nerve following a low calorie diet 6 weeks later. Note the tidemark changes of the extent of the previous oedema. c, Right eye cross-sectional image half way through the optic nerve head. Note the high line indicates the height of the swelling at diagnosis and the green volume reduction from the first scan to the most recent one (in this case 6 weeks). d, Left eye IR image of a swollen optic nerve. Note the difference between a and d, indicating asymmetric papilloedema with worse papilloedema in the left eye. e, Left eye IR image the nerve following a low calorie diet 6 weeks later. Note the tidemark changes of the extent of the previous oedema. f, Left eye cross-sectional image half way through the optic nerve head. Note the high line indicates the height of the swelling at diagnosis and the green volume reduction from the first scan to the most recent one (in this case 6 weeks)

Fig. 5

Optical coherence tomography highlighting worsening of papilloedema. OCT is useful for monitoring of changes in papilloedema. a, Right eye infrared (IR) image of a normal small optic nerve in a patient in IIH with ocular remission. Note the tidemark changes of the extent of the previous oedema. b, Colour photograph of right optic nerve with swelling and haemorrhage with recurrence of symptoms. c, Right eye IR image taken at the same time as b. Note the extent of the oedema and the optic nerve is more visible with the OCT image compared to the photo. d, Right eye cross-sectional image half way through the optic nerve head. Note the high line indicates the height of the swelling at this visit and the red volume increase is from the last OCT scan to the most recent one. e, Left eye IR image of a normal small optic nerve in a patient in IIH with ocular remission. Note the tidemark changes of the extent of the previous oedema. f, Colour photograph of left optic nerve with swelling and cotton wool spot changes with recurrence of symptoms. g, Left eye IR image taken at the same time as f. Note the extent of the oedema and the optic nerve is more visible with the OCT image compared to the photo. h, Left eye cross-sectional image half way through the optic nerve head. Note the high line indicates the height of the swelling at this visit and the red volume increase is from the last OCT scan to the most recent one