Approach

The first step is to eliminate or treat causes of intracranial hypertension. If the diagnosis is IIH, a weight-reduction program can be instituted and, if indicated, pharmacologic therapy can be started with the aim of reversing and preventing loss of vision. Treatment can be given for headache if it persists despite use of intracranial pressure-lowering agents and procedures.[1][46]

Elimination or treatment of other causal factors

Other potential causal factors need to be identified (see Diagnostic approach for more information). If an underlying cause for the intracranial hypertension is found, this should be treated appropriately. Tetracyclines, retinoids, and excessive vitamin A should be discontinued. In the setting of corticosteroid withdrawal, restart corticosteroid therapy and taper slowly.

Weight reduction

Studies show that weight-reduction diets can reduce intracranial pressure and papilledema in patients with IIH.[11][54][56] Patients should aim to lose 5% to 10% of their total body weight, and maintain the weight loss. This is a more realistic goal than other diet plans, so is more likely to be achieved than if the patient aims for greater weight loss. In one prospective cohort study, the improvement in symptoms persisted for 3 months after the patients stopped the diet.[57]

Patients with IIH and class III obesity (body mass index >40) have poor results with dietary therapy and should be considered for bariatric surgery.[58][59] A review of published literature found 56 of 61 patients with IIH had resolution of symptoms following surgery.[60] Surgery also addresses additional obesity-related comorbidities. Due to the risk of complications, surgery should be limited to this patient group only.[59] 

Low-sodium diet

Patients are advised to follow a low-sodium diet and to only drink when thirsty (instead of forcing water intake), because excessive water intake may contribute to increased intracranial pressure. A low-sodium diet and mild fluid restriction may be beneficial for patients with IIH who have orthostatic edema.[61] 

Medical therapy to decrease intracranial pressure

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.

Acetazolamide

  • Acetazolamide works by inhibiting carbonic anhydrase, which causes a reduction in transport of sodium ions across the choroid plexus epithelium, but the mechanism of action is likely multifactorial. Acetazolamide decreases cerebrospinal fluid (CSF) production in humans by 6% to 50%.[62]

  • 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 CSF 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]

Furosemide

  • Treatment can be switched from acetazolamide to furosemide.[71][72][73][74] The dose of furosemide can be increased gradually until the desired effect is obtained. Close monitoring of the serum level of potassium is recommended.

  • Furosemide appears to work both by producing diuresis and reducing sodium transport into the brain.[75]

  • Acetazolamide can be given with furosemide in cases of refractory disease, but close monitoring of serum potassium is necessary.[76][77]

Topiramate

  • This drug has weak carbonic anhydrase activity, and a fortunate adverse effect is weight loss. Topiramate may have efficacy similar to acetazolamide.[55][78]

  • It can be useful for its migraine prophylaxis as well as carbonic anhydrase inhibition. However, topiramate can cause confusion, drowsiness, and, rarely, acute angle closure glaucoma.

Headache

Headaches may transiently improve after a single lumbar puncture, but often remain as a major management problem even after medications have been given to reduce papilledema and CSF pressure.

Pharmacotherapy

Beta-blockers or calcium-channel blockers should be avoided, as they may decrease perfusion of the optic nerve head. Tricyclic antidepressants 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.

Shunting procedures

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 CSF diversion may reduce visual decline and improve visual acuity, 68% patients continue to experience headaches 6 months post procedure.[85] Do not overlook the possibility of analgesic- or caffeine-rebound headache in these patients.

Progressive visual loss

If patients lose vision in spite of maximal medical therapy, surgical treatment by optic nerve sheath fenestration or CSF shunting can be done; both procedures have benefits and risks. 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 with CSF shunting.[1] Late deterioration requiring refenestration appears to be uncommon.

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.

Patients should be evaluated by a neuro-ophthalmologist if available; otherwise, a neurologist should collaborate with an ophthalmologist. The ophthalmologist should assess visual function by perimetry and monitor the degree of papilledema with serial fundus photographs if available (if not, Frisén grading can be used). Visual evoked potential is insensitive for monitoring the loss of vision associated with IIH and should not be used.

Other neurologic symptoms and signs

Prisms can be given for the diplopia of sixth cranial nerve palsy, which usually resolves with treatment. 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. Pulse synchronous tinnitus usually responds to weight loss and medical treatments; in persistent tinnitus due to the transverse venous sinus stenosis related to the increased intracranial pressure, transverse sinus stenting may be indicated.

Pregnancy

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.

Surgery in pregnant women can be done under local anesthesia, so need not be delayed. Pregnant women with IIH do not have an increased rate of spontaneous abortion.[99] Despite the presence of elevated intracranial pressure, neuraxial (spinal and epidural) anesthesia can be used without adverse effects (including uncal herniation) for cesarean section and pain relief during labor, even in patients with CSF diversion devices in situ.[100]

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