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Idiopathic intracranial hypertension

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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.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.
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  • Laboratory tests

  • Diagnostic imaging

  • Assessment of comorbidities

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.
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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.

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  • Acetazolamide

  • Topiramate

  • Non-opioid analgesics

  • Opioids

  • Migraine prophylaxis

  • Management of migraine attacks

4.Nonpharmacologic interventions

Lifestyle modifications: provide lifestyle advice in all patients with headache disorders, as these can have considerable impact on the disease course.
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  • Weight loss

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.

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  • Therapeutic lumbar puncture

  • Neurovascular stenting

6.Surgical interventions

Optic nerve sheath fenestration: perform surgery for the acute management to preserve vision if there is evidence of declining visual function.
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  • CSF diversion shunting

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.
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  • Patients with IIH without papilledema

  • Patients with CSF diversion shunts (evaluation)

  • Patients with CSF diversion shunts (management of headache)

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.
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9.Follow-up and surveillance

Follow-up: obtain and document the following in any patient with papilledema during follow-up:
visual acuity
pupil examination
formal visual field assessment
dilated fundal examination to grade the papilledema
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