Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

all patients

1st line – management of factors that may increase intracranial pressure

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1st line – 

management of factors that may increase intracranial pressure

Once intracranial hypertension has been diagnosed, a weight-reduction program can be instituted in overweight patients, and other presumed causal factors should be eliminated or treated (e.g., discontinue tetracyclines, retinoids, or excessive vitamin A; or, for corticosteroid withdrawal, restart corticosteroid therapy and taper more slowly).[11][54][56][57] Patients are advised to follow a low-sodium diet and to only drink when thirsty (instead of forcing water intake).

For people who are overweight or obese it is recommended to lose 5% to 10% of their total body weight, and maintain the weight loss. Patients with class III obesity (body mass index >40) have poor results with dietary therapy and should be considered for bariatric surgery.[58][59]

There are many associated diseases and iatrogenic associations of intracranial hypertension; these should be eliminated or reduced where possible.[1]

Consider – pharmacotherapy

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Consider – 

pharmacotherapy

Treatment recommended for SOME patients in selected patient group

At initial presentation, many patients are symptomatic, have mild loss of vision, and have a moderate degree of papilledema. In addition to a low-sodium weight-reduction program and the elimination of causal factors, these patients may be started on medical therapy.

Evidence suggests that the use of acetazolamide with a low-sodium weight-reduction diet improves visual field function in patients with IIH and mild visual loss.[54] The combination of acetazolamide and diet has also been shown to significantly improve papilledema grade, quality-of-life measures, and cerebrospinal fluid pressure at 6 months.[54][63] However, one Cochrane review concluded that there was insufficient evidence for the use of acetazolamide and it was unclear whether possible benefits outweighed the increased risk of side effects compared with placebo.[64] The patient should take acetazolamide until symptoms and signs regress, or if side effects interfere with daily activities.[65][66] The maximum tolerated dosage should be taken, which sometimes requires down-titrating until side effects improve and then slowly increasing the titration. Patients with more severe visual loss need a higher initial dosage and a more rapid titration.

Adverse effects of acetazolamide include: changes to the taste of food and drink (which may result in weight loss); tingling in the fingers, toes, and perioral region; malaise; renal stones; metabolic acidosis; hypokalemia (particularly when combined with other diuretics); aplastic anemia (rarely).[54][67][68][69][70]

Treatment can be switched from acetazolamide to furosemide or topiramate, both of which are regarded as secondary treatments.[71][72][73][74] For furosemide, the dose can be increased gradually until the desired effect is obtained. Close monitoring of the serum level of potassium is recommended. Acetazolamide can also be given with furosemide in cases of refractory disease, but close monitoring of serum potassium is necessary.[76][77]

Topiramate may have efficacy similar to acetazolamide.[55][78] It can be useful for its migraine prophylaxis as well as carbonic anhydrase inhibition.

Pregnant women are generally given the same treatments as for nonpregnant patients; however, caution is advised.[96] There are no adequate studies of acetazolamide in pregnant women. It has been shown to be teratogenic in animals, and there have been case reports of congenital malformations, neonatal electrolyte disturbances, and low birth weight in humans.[97] Acetazolamide may be used after the first trimester (e.g., in cases where the patient is losing vision or has high-grade papilledema), but only if the benefits outweigh the potential risks to the fetus. There are no well-controlled studies of furosemide in pregnant women.[98] Again, it should only be used if the benefits outweigh the potential risks to the fetus. Topiramate is not recommended in pregnant women due to an increased risk of cleft lip and/or cleft palate in the fetus, as well as other issues (e.g., small for gestational age, maternal metabolic acidosis, preterm labor).[46] However, there may be circumstances where the use of topiramate is justified because the benefits of treatment outweigh the risks to the fetus. Always discuss the risk and benefits of drug treatments with pregnant women before initiating therapy.

Primary options

acetazolamide: 500 mg orally twice daily initially, increase by 250 mg/day increments every 4 days according to response, maximum 4000 mg/day

Secondary options

furosemide: 20-40 mg orally two to three times daily

OR

topiramate: consult specialist for guidance on dose

OR

acetazolamide: 500 mg orally twice daily initially, increase by 250 mg/day increments every 4 days according to response, maximum 4000 mg/day

and

furosemide: 20-40 mg orally two to three times daily

Plus – analgesia

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persistent headache
Plus – 

analgesia

Treatment recommended for ALL patients in selected patient group

Headaches often remain as a major management problem, even after medications have been given that decrease cerebrospinal fluid pressure.

Tricyclic antidepressants (e.g., amitriptyline) can be used, but because weight gain is an adverse effect, they should be prescribed at a low dosage. They can also cause urinary retention, dry mouth, and sedation, although these are seldom a significant problem.

Nonsteroidal anti-inflammatory drugs, such as naproxen, can also be used, but should be taken only 2 days per week to prevent rebound headache.

Patients with a history of migraine, or analgesic- or caffeine-rebound headaches, may require intravenous dihydroergotamine in combination with metoclopramide. Metoclopramide should be used for up to 5 days only, in order to minimize the risk of neurologic and other adverse effects.[79] It is not uncommon for papilledema to resolve and analgesic-overuse headaches to remain.

Primary options

amitriptyline: 10-50 mg orally once daily at bedtime

OR

naproxen: 250-500 mg orally twice daily when required, maximum 1250 mg/day (maximum 2 days per week)

Secondary options

dihydroergotamine: 1 mg intravenously initially, repeat in 1 hour if no improvement, maximum 2 mg/total dose and 6 mg/week

and

metoclopramide: 5-10 mg intravenously every 8 hours when required for a maximum of 5 days, maximum 30 mg/day

Consider – cerebrospinal fluid shunting

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persistent headache
Consider – 

cerebrospinal fluid shunting

Treatment recommended for SOME patients in selected patient group

Various shunting procedures have been used for intractable headache or failed medical therapy, including lumbar subarachnoid-peritoneal, cisternoatrial, ventriculoatrial, ventriculojugular, and ventriculoperitoneal shunts.[80][81][82][83][84]

The most common complications are shunt occlusion and intracranial hypotension. Shunt malfunction can also be accompanied by severe loss of vision. Less common complications are back pain, abdominal pain, infection of the disk space, meningitis, disconnection of tubing, and descent of the cerebellar tonsils. Although cerebrospinal fluid diversion may reduce visual decline and improve visual acuity, 68% patients continue to experience headaches 6 months post procedure.[85] It is critical not to overlook analgesic- or caffeine-rebound headache in these patients.

Surgery in pregnant women can be performed under local anesthesia, so need not be delayed.

Plus – optic nerve sheath fenestration or cerebrospinal fluid shunting

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progressive visual loss
Plus – 

optic nerve sheath fenestration or cerebrospinal fluid shunting

Treatment recommended for ALL patients in selected patient group

If patients lose vision in spite of full medical therapy, surgical treatment by optic nerve sheath fenestration or cerebrospinal fluid (CSF) shunting can be done. The effectiveness of both techniques has been demonstrated in large case studies.[86][87][88][89][90][91][92][93][94]

The clinical course of optic nerve sheath fenestration awaits further study, as meta-analysis of retrospective case series also shows approximately 10% of patients lose vision despite this procedure;[95] a similar percentage of vision loss occurs in CSF-shunted patients.[1] Late deterioration requiring refenestration appears to be uncommon. Surgery in pregnant women can be done under local anesthesia, so need not be delayed.

Optic nerve sheath fenestration usually produces immediate results, with improvement in vision occurring over several months. The procedure is usually performed on the eye with the worse vision, and often vision recovers and papilledema is decreased in the unoperated eye as well. This improvement in the nonoperated eye can persist for months or even years, but is often less pronounced than in the operated eye. Complications include loss of vision in the perioperative period, ocular motility disorders, and tonic pupils. Loss of vision may be related to a fall in arterial blood pressure.

Plus – optical prisms

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all patients
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with diplopia
Plus – 

optical prisms

Treatment recommended for ALL patients in selected patient group

Prisms can be given for the diplopia of sixth cranial nerve palsy. The diplopia often resolves with treatment.

Plus – nonsteroidal anti-inflammatory drug

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all patients
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with neck pain
Plus – 

nonsteroidal anti-inflammatory drug

Treatment recommended for ALL patients in selected patient group

Nonsteroidal anti-inflammatory drugs can be used for neck pain but should be limited to only 2 days per week if there is concomitant headache.

Primary options

ibuprofen: 400-800 mg orally every 6-8 hours when required, maximum 3200 mg/day

OR

naproxen: 250-500 mg orally twice daily when required, maximum 1250 mg/day

Consider – transverse sinus stenting

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all patients
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persistent pulse synchronous tinnitus
Consider – 

transverse sinus stenting

Treatment recommended for SOME patients in selected patient group

While pulse synchronous tinnitus usually responds to weight loss and medical treatments, in those cases due to the transverse venous sinus stenosis related to the increased intracranial pressure, transverse sinus stenting may be indicated.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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