What are the guideline sources, guidelines and references for Idiopathic intracranial hypertension ?
Guideline sources The following summarized guidelines for the evaluation and management of idiopathic intracranial hypertension are prepared by our editorial team based on guidelines from the Association of British Neurologists (ABN/RCOphth/BASH/SBNS 2018). 1 Guidelines 1. Diagnostic investigations History and physical examination: elicit a careful history to exclude any possible secondary causes of raised intracranial hypertension, recognizing that the causal link with idiopathic intracranial hypertension and a number of diseases and medications is not clear. E Show 4 more Laboratory tests: Obtain a CBC in all patients to exclude anemia in patients with raised intracranial pressure. E https://web.pathway.md/diseases/rec9450FedDYumvPt 1/5 6/24/23, 2:28 PM Idiopathic intracranial hypertension Pathway Consider obtaining other additional blood tests to exclude secondary causes of raised intracranial pressure in patients deemed to be atypical. E Diagnostic imaging: obtain urgent brain MRI within 24 hours in patients with suspected idiopathic intracranial hypertension. Obtain urgent brain CT if MRI is unavailable within 24 hours and subsequent MRI if no lesion is identified. E Show 4 more Assessment of comorbidities: assess for anxiety and depression and address appropriately, as patients with idiopathic intracranial hypertension may have significantly higher levels of anxiety and depression and a lower quality of life probably as a response to chronic pain. E Show 2 more 2. Diagnostic procedures Diagnostic lumbar puncture: perform a lumbar puncture after normal imaging in all patients with papilledema to check opening pressure and ensure that contents are normal. E Show 7 more 3. Medical management General principles: consider providing a multidisciplinary team approach including, ideally, an assessment by an experienced clinician with an interest in headache management. E Acetazolamide: consider initiating acetazolamide (250-500 mg BID with daily dose uptitration) in patients with idiopathic intracranial hypertension symptoms. E Show 2 more Landmark trials: IIHTT In patients with idiopathic intracranial hypertension and mild visual loss who received a low- sodium weight-reduction diet, acetazolamide was superior to placebo with respect to improvement in perimetric mean deviation in the most affected eye at 6 months. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee et al. JAMA. 2014 Apr 23-30. Topiramate: consider initiating topiramate with weekly dose escalation from 25 mg to 50 mg in patients with idiopathic intracranial hypertension. E Show 2 more Non-opioid analgesics: consider initiating short-term analgesics (NSAIDs or acetaminophen) in the first few weeks following diagnosis. Consider preferring indomethacin due to its effect of reducing intracranial pressure. E Show 2 more https://web.pathway.md/diseases/rec9450FedDYumvPt 2/5 6/24/23, 2:28 PM Idiopathic intracranial hypertension Pathway Opioids: Do not prescribe opioids for headaches. D Discontinue opioids gradually with at least 1 month analgesic free to determine effectiveness. E Migraine prophylaxis: consider initiating early migraine preventatives as these can take 3-4 months to reach maximal efficacy. Recognize that migraine prophylaxis is most likely to be effective in patients with settling intracranial pressure and in patients with resolved papilledema (idiopathic intracranial hypertension in ocular remission). E Show 5 more Management of migraine attacks: Consider administering triptan acute therapy in combination with either a NSAID or acetaminophen and an antiemetic with prokinetic properties for migraine attacks, while limiting their use to 2 days/week or a maximum of 10 days/month. E Consider discontinuing triptan medications abruptly or weaning down within a month. E 4. Nonpharmacologic interventions Lifestyle modifications: provide lifestyle advice in all patients with headache disorders, as these can have considerable impact on the disease course. E Show 3 more Weight loss: counsel all patients with a BMI > 30 kg/m about weight management at the earliest opportunity once definite idiopathic intracranial hypertension is diagnosed. E Show 2 more 5. Therapeutic procedures Greater occipital nerve block: insufficient evidence to recommend greater occipital nerve blocks for the management of headaches in patients with newly diagnosed idiopathic intracranial hypertension. I Therapeutic lumbar puncture: avoid performing lumbar punctures for the treatment of headache in patients with newly diagnosed idiopathic intracranial hypertension. D Show 2 more Neurovascular stenting: do not perform neurovascular stenting for the treatment of headache in patients with idiopathic intracranial hypertension. D Show 2 more 6. Surgical interventions https://web.pathway.md/diseases/rec9450FedDYumvPt 3/5 6/24/23, 2:28 PM Idiopathic intracranial hypertension Pathway Optic nerve sheath fenestration: perform surgery for the acute management to preserve vision if there is evidence of declining visual function. E Show 2 more CSF diversion shunting: perform surgery by an experienced clinician, where possible, with an interest in CSF disorders. E Show 4 more 7. Specific circumstances Pregnant patients: insufficient evidence to support the use of acetazolamide during pregnancy. Recognize that manufacturers do not recommend its use. Discuss the risks and benefits regarding the necessity of acetazolamide treatment during pregnancy with the patient because of the possible risk of teratogenic effects. I Show 9 more Patients with IIH without papilledema: manage patients with definite diagnosis of idiopathic intracranial hypertension without papilledema as typical idiopathic intracranial hypertension and counsel about weight management. E Show 2 more Patients with CSF diversion shunts (evaluation): obtain funduscopy in all shunted patients with idiopathic intracranial hypertension presenting with an acute exacerbation of headaches, to establish if papilledema exists and where visual function (including formal visual fields) is documented to be worsening. Consider performing surgical intervention after the evaluation, if needed. Take further care to establish whether the headache is secondary to raised intracranial pressure in patients with atrophic optic nerves. E Show 5 more Patients with CSF diversion shunts (management of headache): do not perform shunt revision in patients without current papilledema or imminent risk to vision. D Show 3 more 8. Patient education General counseling: ensure that all patients recognize that they have been diagnosed with a rare disease and need appropriate support to deal with the psychological burden of living with a chronic condition. E Show 3 more 9. Follow-up and surveillance Follow-up: Obtain and document the following in any patient with papilledema during follow-up: visual acuity https://web.pathway.md/diseases/rec9450FedDYumvPt 4/5 6/24/23, 2:28 PM Idiopathic intracranial hypertension Pathway pupil examination formal visual field assessment dilated fundal examination to grade the papilledema BMI. E Show 5 more References 1. Susan P Mollan, Brendan Davies, Nick C Silver et al. Idiopathic intracranial hypertension: consensus guidelines on management. J Neurol Neurosurg Psychiatry. 2018 Oct;89 10 1088 1100. Open 2. NORDIC Idiopathic Intracranial Hypertension Study Group Writing Committee, Michael Wall, Michael P McDermott et al. Effect of acetazolamide on visual function in patients with idiopathic intracranial hypertension and mild visual loss: the idiopathic intracranial hypertension treatment trial. JAMA. 2014 Apr 23 30;311 16 1641 51. Open https://web.pathway.md/diseases/rec9450FedDYumvPt 5/5