Pseudotumor Cerebri Medication

  • Author: James Goodwin, MD; Chief Editor: Robert A Egan, MD   more...
 
Updated: May 7, 2010
 

Medication Summary

Specific therapy for idiopathic intracranial hypertension (IIH) is aimed at lowering intracranial pressure (ICP) pharmacologically. Carbonic anhydrase inhibitors and other diuretics are thought to have their effect on ICP by reducing cerebral spinal fluid (CSF) production at the choroid plexus. Cardiac glycosides have a similar effect. Corticosteroids are effective in reducing ICP. However, the mechanism of action is unknown. Corticosteroids are often used as maximum medical management when rapid lowering of ICP is required.

Next

Carbonic anhydrase inhibitors

Class Summary

These agents reduce CSF production and lower ICP.

View full drug information

Acetazolamide (Diamox)

 

Reduces CSF production by about 50% (Maren, 1972; McCarthy and Reed, 1974) and lowers ICP. Commonly achieves long-lasting control of transient visual obscurations (TVO), headache, and diplopia, all of which are manifestations of intracranial hypertension, even though papilledema does not resolve completely. Effect on ICP has been shown to be unsustained (Plum and Siesio, 1975), and many patients develop adverse effects severe enough to hinder compliance.

Some clinicians prefer Sequels formulation of Diamox, which may be better tolerated than standard version.

Few patients tolerate more than 2 g/d, but 4 g/d may be required to produce measurable pressure-lowering effect (Gucer and Viernstein, 1978); treatment usually initiated at 1 g/d and increased to 2 g/d if symptoms are not controlled and adverse effects are not severe; treatment with Diamox alone not appropriate for patients who are experiencing progressive visual field loss.

Previous
Next

Cardiac glycosides

Class Summary

These agents reduce CSF production at choroid plexus and reduce ICP.

View full drug information

Digoxin (Lanoxin)

 

Present in high concentration in choroid plexuses of patients taking standard cardiac doses (Bertler, 1973), has been shown to reduce CSF production by as much as 78% in humans (Neblett, 1972), probably by inhibiting Na-K-ATPase pump (Vates, 1963). Only one report in which a patient with IIH was treated with digoxin, but patient was asymptomatic, so not known whether symptoms would have been controlled (Schott and Holt, 1974).

Previous
Next

Glucocorticoids

Class Summary

These agents reduce ICP through an unknown mechanism.

View full drug information

Prednisone (Sterapred)

 

Mechanism of action by which corticosteroids lower CSF pressure unknown. Some believe that may facilitate outflow at arachnoid granulations.

Previous
Proceed to Follow-up
 
 
Contributor Information and Disclosures
Author

James Goodwin, MD  Associate Professor, Departments of Neurology and Ophthalmology, University of Illinois College of Medicine; Director, Neuro-Ophthalmology Service, University of Illinois Eye and Ear Infirmary

James Goodwin, MD is a member of the following medical societies: American Academy of Neurology, Illinois State Medical Society, North American Neuro-Ophthalmology Society, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Eric R Eggenberger, DO, MS, FAAN  Professor, Vice-Chairman, Department of Neurology and Ophthalmology, Colleges of Osteopathic Medicine and Human Medicine, Michigan State University; Director of Michigan State University Ocular Motility Laboratory; Director of National Multiple Sclerosis Society Clinic, Michigan State University

Eric R Eggenberger, DO, MS, FAAN is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, American Osteopathic Association, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Robert A Egan, MD  Director of Neuro-Ophthalmology, St Helena Hospital

Robert A Egan, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, North American Neuro-Ophthalmology Society, and Oregon Medical Association

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Robert A Egan, MD  Director of Neuro-Ophthalmology, St Helena Hospital

Robert A Egan, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, North American Neuro-Ophthalmology Society, and Oregon Medical Association

Disclosure: Nothing to disclose.

References

  1. Jindal M, Hiam L, Raman A, Rejali D. Idiopathic intracranial hypertension in otolaryngology. Eur Arch Otorhinolaryngol. Jun 2009;266(6):803-6. [Medline].

  2. Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology. May 13 2003;60(9):1418-24. [Medline].

  3. Bateman GA. Association between arterial inflow and venous outflow in idiopathic and secondary intracranial hypertension. J Clin Neurosci. Jun 2006;13(5):550-6; discussion 557. [Medline].

  4. Bateman GA. Arterial inflow and venous outflow in idiopathic intracranial hypertension associated with venous outflow stenoses. J Clin Neurosci. Apr 2008;15(4):402-8. [Medline].

  5. 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].

  6. Durcan FJ, Corbett JJ, Wall M. The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch Neurol. Aug 1988;45(8):875-7. [Medline].

  7. Radhakrishnan K, Ahlskog JE, Cross SA, et al. Idiopathic intracranial hypertension (pseudotumor cerebri). Descriptive epidemiology in Rochester, Minn, 1976 to 1990. Arch Neurol. Jan 1993;50(1):78-80. [Medline].

  8. Radhakrishnan K, Thacker AK, Bohlaga NH, et al. Epidemiology of idiopathic intracranial hypertension: a prospective and case-control study. J Neurol Sci. May 1993;116(1):18-28. [Medline].

  9. Agostoni E, Aliprandi A. Alterations in the cerebral venous circulation as a cause of headache. Neurol Sci. May 2009;30 Suppl 1:S7-10. [Medline].

  10. Ney JJ, Volpe NJ, Liu GT, Balcer LJ, Moster ML, Galetta SL. Functional visual loss in idiopathic intracranial hypertension. Ophthalmology. Sep 2009;116(9):1808-1813.e1. [Medline].

  11. Radhakrishnan K, Ahlskog JE, Garrity JA, Kurland LT. Idiopathic intracranial hypertension. Mayo Clin Proc. Feb 1994;69(2):169-80. [Medline].

  12. Kelly SJ, O'Donnell T, Fleming JC, Einhaus S. Pseudotumor cerebri associated with lithium use in an 11-year-old boy. J AAPOS. Apr 2009;13(2):204-6. [Medline].

  13. Rogers AH, Rogers GL, Bremer DL, McGregor ML. Pseudotumor cerebri in children receiving recombinant human growth hormone. Ophthalmology. Jun 1999;106(6):1186-9; discussion 1189-90. [Medline].

  14. Howell SJ, Wilton P, Shalet SM. Growth hormone replacement in patients with Langerhan's cell histiocytosis. Arch Dis Child. May 1998;78(5):469-73. [Medline].

  15. Crock PA, McKenzie JD, Nicoll AM, et al. Benign intracranial hypertension and recombinant growth hormone therapy in Australia and New Zealand. Acta Paediatr. Apr 1998;87(4):381-6. [Medline].

  16. Williams JB. Adverse effects of thyroid hormones. Drugs Aging. Dec 1997;11(6):460-9. [Medline].

  17. Raghavan S, DiMartino-Nardi J, Saenger P, Linder B. Pseudotumor cerebri in an infant after L-thyroxine therapy for transient neonatal hypothyroidism. J Pediatr. Mar 1997;130(3):478-80. [Medline].

  18. Sacchi S, Russo D, Avvisati G, et al. All-trans retinoic acid in hematological malignancies, an update. GER (Gruppo Ematologico Retinoidi). Haematologica. Jan-Feb 1997;82(1):106-21. [Medline].

  19. Visani G, Bontempo G, Manfroi S, et al. All-trans-retinoic acid and pseudotumor cerebri in a young adult with acute promyelocytic leukemia: a possible disease association. Haematologica. Mar-Apr 1996;81(2):152-4. [Medline].

  20. Selleri C, Pane F, Notaro R, et al. All-trans-retinoic acid (ATRA) responsive skin relapses of acute promyelocytic leukaemia followed by ATRA-induced pseudotumour cerebri. Br J Haematol. Mar 1996;92(4):937-40. [Medline].

  21. Ahmad S. Amiodarone and reversible benign intracranial hypertension. Cardiology. Jan-Feb 1996;87(1):90. [Medline].

  22. Varadi G, Lossos A, Or R, et al. Successful allogeneic bone marrow transplantation in a patient with ATRA-inducedpseudotumor cerebri. Am J Hematol. Oct 1995;50(2):147-8. [Medline].

  23. Sivin I. Serious adverse events in Norplant users reported to the Food and Drug Administration's MedWatch Spontaneous Reporting System. Obstet Gynecol. Aug 1995;86(2):318-20. [Medline].

  24. Baqui AH, de Francisco A, Arifeen SE, et al. Bulging fontanelle after supplementation with 25,000 IU of vitamin A in infancy using immunization contacts. Acta Paediatr. Aug 1995;84(8):863-6. [Medline].

  25. Alder JB, Fraunfelder FT, Edwards R. Levonorgestrel implants and intracranial hypertension. N Engl J Med. Jun 22 1995;332(25):1720-1. [Medline].

  26. Malozowski S, Tanner LA, Wysowski DK, et al. Benign intracranial hypertension in children with growth hormone deficiency treated with growth hormone. J Pediatr. Jun 1995;126(6):996-9. [Medline].

  27. Campos SP, Olitsky S. Idiopathic intracranial hypertension after L-thyroxine therapy for acquired primary hypothyroidism. Clin Pediatr (Phila). Jun 1995;34(6):334-7. [Medline].

  28. Nasr SZ, Schaffert D. Symptomatic increase in intracranial pressure following pancreatic enzyme replacement therapy for cystic fibrosis. Pediatr Pulmonol. Jun 1995;19(6):396-7. [Medline].

  29. Price DA, Clayton PE, Lloyd IC. Benign intracranial hypertension induced by growth hormone treatment. Lancet. Feb 18 1995;345(8947):458-9. [Medline].

  30. Kesler A, Yatziv Y, Shapira I, et al. Increased red blood cell aggregation in patients with idiopathic intracranial hypertension. A hitherto unexplored pathophysiological pathway. Thromb Haemost. Oct 2006;96(4):483-7. [Medline].

  31. Warner JE, Larson AJ, Bhosale P, Digre KB, Henley C, Alder SC. Retinol-binding protein and retinol analysis in cerebrospinal fluid and serum of patients with and without idiopathic intracranial hypertension. J Neuroophthalmol. Dec 2007;27(4):258-62. [Medline].

  32. Fraser JA, Bruce BB, Rucker J, Fraser LA, Atkins EJ, Newman NJ, et al. Risk factors for idiopathic intracranial hypertension in men: a case-control study. J Neurol Sci. Mar 15 2010;290(1-2):86-9. [Medline].

  33. Nampoory MR, Johny KV, Gupta RK, et al. Treatable intracranial hypertension in patients with lupus nephritis. Lupus. 1997;6(7):597-602. [Medline].

  34. Leker RR, Steiner I. Anticardiolipin antibodies are frequently present in patients with idiopathic intracranial hypertension. Arch Neurol. Jun 1998;55(6):817-20. [Medline].

  35. Sussman J, Leach M, Greaves M, et al. Potentially prothrombotic abnormalities of coagulation in benign intracranial hypertension. J Neurol Neurosurg Psychiatry. Mar 1997;62(3):229-33. [Medline].

  36. Bachman DT, Srivastava G. Emergency department presentations of Lyme disease in children. Pediatr Emerg Care. Oct 1998;14(5):356-61. [Medline].

  37. Agid R, Farb RI, Willinsky RA, et al. Idiopathic intracranial hypertension: the validity of cross-sectional neuroimaging signs. Neuroradiology. Aug 2006;48(8):521-7. [Medline].

  38. Stone MB. Ultrasound diagnosis of papilledema and increased intracranial pressure in pseudotumor cerebri. Am J Emerg Med. Mar 2009;27(3):376.e1-376.e2. [Medline].

  39. Burgett RA, Purvin VA, Kawasaki A. Lumboperitoneal shunting for pseudotumor cerebri. Neurology. Sep 1997;49(3):734-9. [Medline].

  40. Woodworth GF, McGirt MJ, Elfert P, et al. Frameless stereotactic ventricular shunt placement for idiopathic intracranial hypertension. Stereotact Funct Neurosurg. 2005;83(1):12-6. [Medline].

  41. Goh KY, Schatz NJ, Glaser JS. Optic nerve sheath fenestration for pseudotumor cerebri. J Neuroophthalmol. Jun 1997;17(2):86-91. [Medline].

  42. Chandrasekaran S, McCluskey P, Minassian D, Assaad N. Visual outcomes for optic nerve sheath fenestration in pseudotumour cerebri and related conditions. Clin Experiment Ophthalmol. Sep 2006;34(7):661-5. [Medline].

  43. Spoor TC, McHenry JG. Long-term effectiveness of optic nerve sheath decompression for pseudotumor cerebri. Arch Ophthalmol. May 1993;111(5):632-5. [Medline].

  44. Nithyanandam S, Manayath GJ, Battu RR. Optic nerve sheath decompression for visual loss in intracranial hypertension: Report from a tertiary care center in South India. Indian J Ophthalmol. Mar-Apr 2008;56(2):115-20. [Medline].

  45. Feldon SE. Visual outcomes comparing surgical techniques for management of severe idiopathic intracranial hypertension. Neurosurg Focus. 2007;23(5):E6. [Medline].

  46. Bussière M, Falero R, Nicolle D, Proulx A, Patel V, Pelz D. Unilateral Transverse Sinus Stenting of Patients with Idiopathic Intracranial Hypertension. AJNR Am J Neuroradiol. Nov 26 2009;[Medline].

  47. Arac A, Lee M, Steinberg GK, Marcellus M, Marks MP. Efficacy of endovascular stenting in dural venous sinus stenosis for the treatment of idiopathic intracranial hypertension. Neurosurg Focus. Nov 2009;27(5):E14. [Medline].

  48. Fridley J, Foroozan R, Sherman V, Brandt ML, Yoshor D. Bariatric surgery for the treatment of idiopathic intracranial hypertension. J Neurosurg. Jan 22 2010;[Medline].

  49. Rowe FJ, Sarkies NJ. Assessment of visual function in idiopathic intracranial hypertension: a prospective study. Eye. 1998;12 (Pt 1):111-8. [Medline].

  50. Kupersmith MJ, Gamell L, Turbin R, et al. Effects of weight loss on the course of idiopathic intracranial hypertension in women. Neurology. Apr 1998;50(4):1094-8. [Medline].

  51. Wall M, George D. Visual loss in pseudotumor cerebri. Incidence and defects related to visual field strategy. Arch Neurol. Feb 1987;44(2):170-5. [Medline].

  52. Shah VA, Kardon RH, Lee AG, Corbett JJ, Wall M. Long-term follow-up of idiopathic intracranial hypertension: the Iowa experience. Neurology. Feb 19 2008;70(8):634-40. [Medline].

  53. Sugerman HJ, Felton WL 3rd, Salvant JB Jr. Effects of surgically induced weight loss on idiopathic intracranial hypertension in morbid obesity. Neurology. Sep 1995;45(9):1655-9. [Medline].

  54. Sugerman HJ, Felton WL 3rd, Sismanis A, et al. Gastric surgery for pseudotumor cerebri associated with severe obesity. Ann Surg. May 1999;229(5):634-40; discussion 640-2. [Medline].

  55. Digre KB, Varner MW, Corbett JJ. Pseudotumor cerebri and pregnancy. Neurology. Jun 1984;34(6):721-9. [Medline].

  56. Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology. Feb 1991;41(2 (Pt 1)):239-44. [Medline].

  57. Beller GA, Smith TW, Abelmann WH, et al. Digitalis intoxication. A prospective clinical study with serum level correlations. N Engl J Med. May 6 1971;284(18):989-97. [Medline].

  58. Bertler A, Andersson KE, Wettrell G. Letter: Concentration of digoxin in choroid plexus. Lancet. Dec 22 1973;2(7843):1453-4. [Medline].

  59. Blethen SL, Allen DB, Graves D, et al. Safety of recombinant deoxyribonucleic acid-derived growth hormone: The National Cooperative Growth Study experience. J Clin Endocrinol Metab. May 1996;81(5):1704-10. [Medline].

  60. Chung EK. Digitalis intoxication. Amsterdam: Excerpta Medical Foundation; 1969.

  61. Cruz OA, Fogg SG, Roper-Hall G. Pseudotumor cerebri associated with cyclosporine use. Am J Ophthalmol. Sep 1996;122(3):436-7. [Medline].

  62. Gucer G, Viernstein L. Long-term intracranial pressure recording in the management of pseudotumor cerebri. J Neurosurg. Aug 1978;49(2):256-63. [Medline].

  63. Maren TH. Bicarbonate formation in cerebrospinal fluid: role in sodium transport and pH regulation. Am J Physiol. Apr 1972;222(4):885-99. [Medline].

  64. McCarthy KD, Reed DJ. The effect of acetazolamide and furosemide on cerebrospinal fluid production and choroid plexus carbonic anhydrase activity. J Pharmacol Exp Ther. Apr 1974;189(1):194-201. [Medline].

  65. Neblett CR, Waltz TA, McNeel DP, et al. Effect of cardiac glycosides on human cerebrospinal-fluid production. Lancet. Nov 11 1972;2(7785):1008-9. [Medline].

  66. Physicans Desk Reference. Physicans Desk Reference. ed. Medical Economics Data, US; 1997:1679.

  67. Plum F, Siesjo BK. Recent advances in CSF physiology. Anesthesiology. Jun 1975;42(6):708-730. [Medline].

  68. Querfeld U. Benign intracranial hypertension (pseudotumour cerebri) during rhGH therapy. Br J Clin Pract Suppl. Aug 1996;85:68. [Medline].

  69. Schott GD, Holt D. Letter: Digoxin in benign intracranial hypertension. Lancet. Mar 2 1974;1(7853):358-9. [Medline].

  70. Smith TW, Haber E. Digitalis. I. N Engl J Med. Nov 1 1973;289(18):945-52. [Medline].

  71. Smith TW, Willerson JT. Suicidal and accidental digoxin ingestion. Report of five cases with serum digoxin level correlations. Circulation. Jul 1971;44(1):29-36. [Medline].

  72. Vates TS, Bonting SL, Oppelt WW. Na-K activated adenosine triphosphatase and formation of cerebrospinal fluid in the cat. Am J Physiol. May 1964;206:1165-72. [Medline].

  73. Visani G, Manfroi S, Tosi P, Martinelli G. All-trans-retinoic acid and pseudotumor cerebri. Leuk Lymphoma. Nov 1996;23(5-6):437-42. [Medline].

  74. Wilkes BN, Siatkowski RM. Progressive optic neuropathy in idiopathic intracranial hypertension after optic nerve sheath fenestration. J Neuroophthalmol. Dec 2009;29(4):281-3. [Medline].

 
Previous
Next
 
Left optic disc with moderate chronic papilledema in a patient with pseudotumor cerebri. Paton lines (arc-shaped retinal wrinkles concentric with disc margin) are seen along the temporal side of the inferior pole of the disc.
Right optic disc with postpapilledema optic atrophy in a patient with pseudotumor cerebri. Diffuse pallor of the disc and absence of small arterial vessels on the surface are noted, with very little disc elevation. The disc margin at the upper and lower poles and nasally is obscured by some residual edema in the nerve fiber layer and gliosis that often persists even after all the edema has resolved.
The most common early visual field defect in papilledema as the optic nerve develops optic atrophy is an inferior nasal defect as shown in the left eye field chart (left side of figure). The shaded area indicates the defective portion of the field. Note the sharp line of demarcation between defective lower nasal quadrant and normal upper nasal quadrant along the horizontal midline. This is characteristic of early papilledema optic atrophy and is called a nasal step or inferonasal step.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.