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Idiopathic Intracranial Hypertension: Treatment & Medication
Updated: Aug 18, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
The treatment goal for patients is to preserve optic nerve function while managing their increased intracranial pressure.
- Optic nerve function should be carefully monitored with an assessment of visual acuity, color vision, optic nerve head appearance, and perimetry.
- The medical management is multifaceted and consists of the following:
- Weight control for obese patients
- Most patients with this disorder are females who are overweight. Weight loss is a cornerstone in the management of these patients. Unfortunately, weight reduction generally proves to be a difficult task for these patients.16
- As little as a 5-10% weight loss has been demonstrated to result in a reduction of the intracranial pressure with the accompanying resolution of papilledema.5
- To formalize the process of weight reduction, referral to a dietitian is appropriate.
- Treatment of related underlying diseases (see Causes)
- Cessation of exogenous agents related to increased intracranial pressure
- Use of diuretics to control the intracranial pressure
- To protect the optic nerve function, the intracranial pressure must be lowered.
- Acetazolamide appears to be the most effective diuretic in lowering the intracranial pressure. The initial dose should be 0.5-1 g/d. Although for compliance purposes, the 500 mg sequel taken orally twice a day is preferred; some insurers only cover an oral dose of 250 mg taken 4 times per day. Most patients respond to a dose of 1-2 g/d. Although this dose can be increased up to 3-4 g/d, most patients do not tolerate the troubling adverse effects (eg, extremity paresthesias, fatigue, metallic taste when drinking carbonated beverages, decreased libido) of this medication at this high dose.5,1
- In the event of intolerance to acetazolamide, furosemide may be used as a replacement diuretic in this group. Unfortunately, furosemide does not appear to be as effective as acetazolamide.
- Corticosteroids
- Corticosteroids are effective in lowering the intracranial pressure in those patients with an inflammatory etiology for their idiopathic intracranial hypertension.
- In addition, steroids may be used as a supplement to acetazolamide to hasten recovery in patients who present with severe papilledema.
- Because of the significant adverse effects, corticosteroids should not be considered as a long-term solution for these patients. In addition, a rebound in the intracranial pressure may occur during the taper of the corticosteroids.1
- Weight control for obese patients
Surgical Care
Patients with idiopathic intracranial hypertension should be closely monitored while on medical treatment. The frequency of visits is determined by the initial state of the patient's visual function and the response to medical treatment. Despite close follow-up care and maximum medical treatment, some patients experience deterioration of their visual function. In this situation, surgical intervention should be considered. Two procedures that can be performed are optic nerve sheath fenestration or a cerebrospinal fluid diversion procedure (ie, lumboperitoneal shunt, ventriculoperitoneal shunt). Treatment of this disorder by repeated lumbar punctures is considered to be of historic interest.
- Optic nerve sheath fenestration
- Optic nerve sheath fenestration has been demonstrated to result in the reversal of optic nerve edema with some recovery of optic nerve function. In addition, it may result in the decrease in the headache of many patients. The approach to the optic nerve may be from the medial or lateral aspect of the orbit; each technique has its benefits and drawbacks.17
- Occasionally, a bilateral curative effect of the papilledema occurs from unilateral surgery. However, if this is not the case, then the opposite nerve must undergo the same procedure.
- Although the intracranial pressure remains elevated in these patients postoperatively, the local filtering effect of the fenestration acts as a safety valve and eliminates the pressure from being transmitted to the optic nerve.
- Complications related to this procedure include diplopia, optic nerve injury, vascular occlusion, a tonic pupil, and the inherent risk of hemorrhage and infection with intraconal surgery.
- Unfortunately, Spoor has demonstrated that the long-term success rate of this operation may be only 16%.18
- Cerebrospinal fluid diversion procedures (ie, lumboperitoneal shunt, ventriculoperitoneal shunt)
- These two neurosurgical interventions are highly effective in lowering the intracranial pressure. In some facilities, they remain the procedures of choice for treating patients with idiopathic intracranial hypertension who do not respond to maximum medical treatment.1,3,17
- Shunts are also indicated in the following: patients with intractable headaches, regions where no access is available to a surgeon who is comfortable with optic nerve sheath fenestration, and patients with a failed optic nerve sheath fenestration.
Diet
- Weight reduction has been clearly demonstrated to be an important factor in the long-term management of these patients.
- As little as a 5-10% decrease in the total body weight can result in the resolution of papilledema. Unfortunately, weight loss in patients who are obese is difficult.5
- A referral to a dietitian is worthwhile in patients who are motivated to lose weight.
Medication
The medications used in this disorder are directed at lowering intracranial pressure. The diuretic acetazolamide is the most effective drug for this task. Furosemide may be used as a replacement, although it is not as potent as acetazolamide. Corticosteroids are indicated on a short-term basis in patients who present with severe papilledema and compromise of their visual function.
Carbonic anhydrase inhibitors
Carbonic anhydrase (CA) is an enzyme found in many tissues. Catalyzes a reversible reaction where carbon dioxide becomes hydrated and carbonic acid becomes dehydrated. These changes may result in a decrease in cerebrospinal fluid by the choroid plexus.
Acetazolamide (Diamox, Diamox Sequels)
Nonbacteriostatic sulfonamide; potent CA inhibitor, which is effective in diminishing fluid secretion. Lowers intracranial pressure by decreasing production of cerebrospinal fluid. Inhibition of CA results in a drop in sodium ion transport across the choroidal epithelium. Reduction of cerebrospinal fluid production occurs within hours.
Adult
500 mg sequels PO bid; up to 2,000 mg PO bid
Alternatively, 250 mg tab PO qid
Pediatric
5-10 mg/kg PO qid
Can decrease therapeutic levels of lithium and alter excretion of drugs (eg, amphetamines, quinidine, phenobarbital, salicylates) by alkalinizing urine
Documented hypersensitivity; hepatic disease; severe renal disease; adrenocortical insufficiency; severe pulmonary obstruction
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in severe pulmonary disease or in those patients taking high doses of aspirin; adverse reactions may include drowsiness, paresthesias, anaphylaxis, Steven-Johnson syndrome, rash, crystalluria, renal calculus (patients are advised to drink sufficient amounts of water during the day), bone marrow depression, thrombocytopenic purpura, hemolytic anemia, leukopenia, pancytopenia, and agranulocytosis
Loop diuretics
These agents inhibit reabsorption of sodium in the ascending limb of Henle loop and have a weak inhibitory action on carbonic anhydrase.
Furosemide (Lasix)
Unclear how it inhibits cerebrospinal fluid production. A combination of CA inhibition and effect on sodium absorption across the choroid plexus may result in the decrease of cerebrospinal fluid production.
Adult
20-40 mg PO bid initially; may increase by 20 mg to maximum 80 mg PO bid with appropriate monitoring
Pediatric
Not established
Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently with this medication
Documented hypersensitivity; hepatic coma; anuria; severe electrolyte depletion
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Perform frequent serum electrolyte, carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
More on Idiopathic Intracranial Hypertension |
| Overview: Idiopathic Intracranial Hypertension |
| Differential Diagnoses & Workup: Idiopathic Intracranial Hypertension |
Treatment & Medication: Idiopathic Intracranial Hypertension |
| Follow-up: Idiopathic Intracranial Hypertension |
| References |
| Further Reading |
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References
Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. Jun 2004;24(2):138-45. [Medline].
Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. Nov 26 2002;59(10):1492-5. [Medline].
Miller NR, Newman NJ. Pseudotumor cerebri (benign intracranial hypertension). In: Walsh and Hoyt's Clinical Neuro-Ophthalmology. Vol 1. 5th ed. 1999:523-38.
Bateman GA, Stevens SA, Stimpson J. A mathematical model of idiopathic intracranial hypertension incorporating increased arterial inflow and variable venous outflow collapsibility. J Neurosurg. Mar 2009;110(3):446-56. [Medline].
Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. Mar 2008;8(2):87-93. [Medline].
Corbett JJ. The first Jacobson Lecture. Familial idiopathic intracranial hypertension. J Neuroophthalmol. Dec 2008;28(4):337-47. [Medline].
Daniels AB, Liu GT, Volpe NJ, et al. Profiles of obesity, weight gain, and quality of life in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol. Apr 2007;143(4):635-41. [Medline].
Digre KB, Nakamoto BK, Warner JE, Langeberg WJ, Baggaley SK, Katz BJ. A comparison of idiopathic intracranial hypertension with and without papilledema. Headache. Feb 2009;49(2):185-93. [Medline].
Corbett JJ, Savino PJ, Thompson HS, et al. Visual loss in pseudotumor cerebri. Follow-up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss. Arch Neurol. Aug 1982;39(8):461-74. [Medline].
Ney JJ, Volpe NJ, Liu GT, Balcer LJ, Moster ML, Galetta SL. Functional Visual Loss in Idiopathic Intracranial Hypertension. Ophthalmology. Jul 28 2009;[Medline].
Bruce BB, Kedar S, Van Stavern GP, et al. Idiopathic intracranial hypertension in men. Neurology. Jan 27 2009;72(4):304-9. [Medline].
Jiraskova N, Rozsival P. Idiopathic intracranial hypertension in pediatric patients. Clin Ophthalmol. Dec 2008;2(4):723-6. [Medline].
Gonzalez-Hernandez A, Fabre-Pi O, Diaz-Nicolas S, Lopez-Fernandez JC, Lopez-Veloso C, Jimenez-Mateos A. [Headache in idiopathic intracranial hypertension]. Rev Neurol. Jul 1-15 2009;49(1):17-20. [Medline].
Mollan SP, Ball AK, Sinclair AJ, et al. Idiopathic intracranial hypertension associated with iron deficiency anaemia: a lesson for management. Eur Neurol. 2009;62(2):105-8. [Medline].
Lin A, Foroozan R, Danesh-Meyer HV, De Salvo G, Savino PJ, Sergott RC. Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension. Ophthalmology. Dec 2006;113(12):2281-4. [Medline].
Johnson LN, Krohel GB, Madsen RW, March GA Jr. The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri). Ophthalmology. Dec 1998;105(12):2313-7. [Medline].
Brazis PW. Clinical review: the surgical treatment of idiopathic pseudotumour cerebri (idiopathic intracranial hypertension). Cephalalgia. Dec 2008;28(12):1361-73. [Medline].
Spoor TC, McHenry JG. Long-term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. May 1993;111(5):632-5. [Medline].
Further Reading
Related eMedicine topics
Pseudotumor Cerebri
Pseudotumor Cerebri, Pediatric Perspective
Papilledema
Optic Neuropathy, Anterior Ischemic
Clinical guidelines
Diagnosis and treatment of headache.
ACR Appropriateness Criteria® orbits, vision, and visual loss.
Clinical trials
Effect of Ketamine (Ketalar) on Intracranial Pressure
Keywords
idiopathic intracranial hypertension, IIH, papilledema, pseudotumor cerebri, PTC, benign intracranial hypertension, BIH, elevated intracranial pressure, increased intracranial pressure, ICP, optic neuropathy
Treatment & Medication: Idiopathic Intracranial Hypertension