Idiopathic intracranial hypertension: consensus guidelines on management

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).MethodsBetween 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.ResultsOver 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.ConclusionsIn collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.

Raised intracranial pressure in those presenting with headache

“…Measure blood pressure to exclude malignant hypertension, a rare but treatable cause of raised ICP 131920…”

“…Presence of spontaneous venous pulsation at the optic nerve head may be reassuring (video 2). Where there is diagnostic difficulty in detecting papilloedema—eg, mild disc swelling, suspicion of a lumpy optic disc (drusen), or myopic tilted disc, prioritise a second opinion to avoid invasive investigations (such as neuroimaging and lumbar puncture) in the normal patient 131920…”

“…Venography can exclude cerebral venous sinus thrombosis. After an intracranial structural lesion is excluded on neuroimaging, it is deemed safe to proceed to a lumbar puncture (in the lateral decubitus position) to record the opening pressure and send cerebrospinal fluid for analysis to exclude infective, malignant, and inflammatory causes 1920…”

“…It is useful to note that visual fields to confrontation is a gross test, so secondary care doctors must seek formal visual fields at the earliest opportunity 1319. High risk patients are those with established or impending visual loss.…”

“…Initially specialist services, such as neurology or neurosurgical teams, will guide care but some conditions may require ongoing joint management in primary care. For example, patients with idiopathic intracranial hypertension can have chronic headaches with migrainous phenotype which will require input from their family doctors 1920…”