eMedicine Specialties > Neurology > Headache and Pain

Pseudotumor Cerebri: Follow-up

Author: James Goodwin, MD, Director of Neuro-Ophthalmology, Associate Professor, Departments of Neurology and Ophthalmology, University of Illinois College of Medicine
Contributor Information and Disclosures

Updated: May 22, 2008

Follow-up

Further Inpatient Care

  • Admission for pain management
    • Even for initial diagnosis, most patients do not require inpatient care, as lumbar puncture usually is performed in the ambulatory care setting.
    • An occasional patient may develop intractable low-tension headache following lumbar puncture and may require a short hospital stay for intravenous (IV) hydration and analgesic management.
    • A blood patch (by an anesthesiologist) sometimes is indicated if the post-lumbar puncture headache does not subside spontaneously within a few days.
  • Admission for surgical management of increased ICP
    • Patients who complain of progressive visual loss (typically constriction of peripheral vision and/or dimming of vision in one or both eyes) and have documented new visual field loss may respond to high-dose corticosteroids; they should be admitted to the hospital with daily monitoring of visual function.
    • If the visual field worsens or does not recover promptly (ie, within 24-48 h) with corticosteroids, then emergency CSF shunting (lumboperitoneal or ventriculoperitoneal/atrial) or optic nerve sheath fenestration should be carried out.
    • If any delay in implementing surgical decompression of the failing optic nerve is anticipated, then the patient should be moved to the ICU or a "step-down" unit for lumbar CSF drainage until the definitive procedure can be performed. Another short-term treatment option is IV mannitol, but definitive pressure-lowering surgery must be done within 2-3 days.
    • A very small number of patients with normal visual fields may require surgical relief of CSF pressure because of intractable headache. Optic nerve sheath fenestration does not provide reliable CSF pressure normalization or headache relief, and thus these patients require one of the shunting procedures outlined in Surgical Care.

Further Outpatient Care

  • Office visits for visual field examination
    • Patients with normal visual field or stable minor field loss (inferonasal step with little concentric constriction of isopters) can be managed with office visits approximately every 3 months.
    • The interval between visits should be shortened if the patient has any questionable symptoms of vision worsening or if visual field examination reveals a minor or questionably significant new deficit.

Inpatient & Outpatient Medications

  • Acetazolamide: Most patients have adequate relief of symptoms, typically headache, using this first-line agent. Patients typically respond and tolerate brand name Diamox better than the generic form, acetazolamide. Please note that many physicians start patients on 250 mg bid, which is considered too small of a dose — 500 mg bid is the beginning dosage in this condition in adults.
  • Headache prophylaxis
    • For patients with stable visual function but inadequate headache relief with first-line pressure-lowering drugs, treatment with primary headache prophylaxis should be considered.
    • Patients with IIH and headaches with many features of migraine have been encountered. Headaches often can be controlled with amitriptyline, propranolol (Inderal), or any of the other commonly prescribed migraine prophylaxis agents.
  • Corticosteroids/digoxin/furosemide
    • Patients experiencing progressive loss of visual field in one or both eyes should be placed immediately on a high dose (60-100 mg/d) of oral prednisone (or equivalent corticosteroid treatment). Digoxin and furosemide (Lasix) have been advocated by some investigators, but these are on the same effectiveness level as acetazolamide and are not appropriate as sole therapy for patients who are losing vision.
    • If a moderately severe new visual field loss is detected on a routine office visit and the patient is not experiencing progressive symptoms, outpatient management can continue. However, visual fields should be measured every few days or at 1- to 2-week intervals depending on the magnitude and progression of the defect. If the visual field continues to worsen on corticosteroid treatment, the patient should be admitted for immediate surgical management.
    • If the patient presents with symptomatic deterioration of vision, and the examination documents worsening of visual field despite adequate standard medical therapy, the patient should be started immediately on corticosteroids as previously outlined.
      • The patient also should be admitted to the hospital for consideration of emergency surgical decompression.
      • Visual field examination should be performed daily, and surgical decompression should be carried out if no improvement or further worsening is noted in the subsequent 24-48 hours.

Complications

  • Optic atrophy
    • The only severe and permanent complication of IIH is progressive blindness from postpapilledema optic atrophy.
    • As optic nerve axons die, the apparent degree of papilledema may diminish, giving a false sense of improvement. For this reason (and others), the patients must be monitored with frequent visual field examinations.
    • The earliest visual loss is in the peripheral fields (outside 30 degrees) and thus Goldmann-type dynamic perimetry is preferred over computerized, automated visual fields.
    • For reasons that are not clear, the earliest field loss tends to be in the inferior nasal quadrant.
    • Visual acuity and color vision are not affected until late in the disease, when the peripheral visual field isopters are quite contracted.

Prognosis

  • Visual loss
    • Depending on the referral population and the rigor with which visual function is tested, the prognosis for visual loss in IIH has varied considerably in different series. Authors writing in the 1960s and 1970s indicated that fewer than 25% of these patients had functionally significant blindness; however, more recently that figure has been revised upward.
    • In a unique major prospective study of visual function in IIH, Wall and George found that 96% of the 50 patients in a series had some degree of visual field loss on Goldmann-type perimetry, while 92% had abnormal findings on automated perimetry; 50% had abnormal contrast sensitivity and 22% abnormal Snellen visual acuity. During follow-up (2-39 mo, average 12.4 mo), visual fields improved in 60% of patients and deteriorated in 10%.41
    • The University of Iowa observed 20 patients with IIH for more than 10 years. Their studies indicated that 11 had followed a stable course without change in visual field or papilledema and 9 worsened after a stable course for an interval. In 6 out of the 9, the worsening occurred late (28-135 mo after initial presentation) and 3 of the 9 had recurrence after resolution of papilledema that occurred from 12-78 months after initial resolution of papilledema.42

Patient Education

  • Weight management
    • The physician must educate patients concerning the potential for dire consequences in terms of disabling blindness. The importance of weight loss as the only effective means of reducing the papilledema and with it, the threat of progressive blindness, cannot be overemphasized.40,39
    • Patients should be urged to enroll in an aggressive weight-loss program, ideally one using a multidisciplinary approach, including diet and exercise along with psychological and lifestyle counseling.
    • Despite these measures, many patients cannot sustain significant weight reduction and may even require drastic steps such as gastric stapling or resection. These measures can be effective for patients who lose vision despite aggressive medical and surgical management.43,44

Miscellaneous

Medicolegal Pitfalls

  • Visual loss in one or both eyes can evolve rapidly despite the best efforts to arrest the process. In this author's experience, IIH has been the most frequent cause of litigation encountered. Almost uniformly, the cases center on the delay of maximum medical and surgical management beyond what has to be considered ideal and standard practice in the United States for patients who present with rapidly declining vision.
  • The exact time window within which vision loss can be reversed after symptomatic decline is not known. Opinions among experts in the field vary as to how rapidly and aggressively any given patient should have been treated. Usually erring on the side of rapid intervention (hours to days) in such patients is better.
  • This is a dramatic opportunity to save vision that can be easily lost. A major medicolegal pitfall is created when poor outcome is coupled with the perception of delayed treatment.

Special Concerns

  • One of the standard teachings has been that pregnancy exacerbates or triggers the onset of symptomatic IIH.
  • However, at present little statistical evidence exists of a causal association between the two conditions, beyond the fact that both events are common in the age group and gender that is predominantly affected by the disease.45,46
 


More on Pseudotumor Cerebri

Overview: Pseudotumor Cerebri
Differential Diagnoses & Workup: Pseudotumor Cerebri
Treatment & Medication: Pseudotumor Cerebri
Follow-up: Pseudotumor Cerebri
Multimedia: Pseudotumor Cerebri
References
[ CLOSE WINDOW ]

References

  1. 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].

  2. 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].

  3. 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].

  4. 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].

  5. 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].

  6. 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].

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

  8. 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].

  9. 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].

  10. 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].

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

  12. 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].

  13. 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].

  14. 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].

  15. 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].

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

  17. 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].

  18. 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].

  19. 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].

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

  21. 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].

  22. 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].

  23. 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].

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

  25. 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].

  26. 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].

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

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

  29. 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].

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

  31. 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].

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

  33. 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].

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

  35. 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].

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

  37. 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].

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

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

  40. 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].

  41. 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].

  42. 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].

  43. 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].

  44. 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].

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

  46. 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].

  47. 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].

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

  49. 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].

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

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

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

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

  54. 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].

  55. 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].

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

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

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

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

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

  61. 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].

  62. 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].

  63. 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].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

idiopathic intracranial hypertension, IIH, benign intracranial hypertension, pseudotumor cerebri, elevated intracranial pressure, ICP, papilledema, progressive optic atrophy, blindness, cerebral edema, occult cerebral venous outflow abnormalities, vision loss, vision impairment

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

James Goodwin, MD, Director of Neuro-Ophthalmology, Associate Professor, Departments of Neurology and Ophthalmology, University of Illinois College of Medicine
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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing 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.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
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: Nothing to disclose.

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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.