Idiopathic Intracranial Hypertension Clinical Presentation

  • Author: Mark S Gans, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 5, 2012
 

History

  • Patients usually present with symptoms related to increased intracranial pressure. These symptoms include headache, transient visual obscurations, and diplopia due to unilateral or bilateral sixth nerve palsy. Rarely, patients presenting with increased intracranial pressure with related optic nerve edema may be asymptomatic.
  • Nonspecific symptoms may include dizziness, nausea, vomiting, photopsias, retrobulbar pain, and pulse-synchronous tinnitus.[5]
  • Headaches[13]
    • Headaches are recorded in almost all patients with idiopathic intracranial hypertension.
    • The pain is generally described as being diffuse, which worsens in the morning and is exacerbated by the Valsalva maneuver.
  • Transient visual obscurations: This visual symptom occurs in most patients. The disturbance can last up to 30 seconds and is described as a graying out of vision on a monocular or binocular basis. Orthostatic changes, such as standing up or bending over, can induce this symptom.
  • Diplopia: Patients who present with double vision most frequently complain of horizontal displacement of the images. Vertical diplopia is rare, but it has been reported.
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Physical

  • The most significant finding in patients with this disease is bilateral disc edema secondary to the increased intracranial pressure.
  • This papilledema varies from patient to patient and is indistinguishable from optic nerve swelling caused by intracranial space-occupying lesions. In more pronounced cases of disc swelling, macular involvement with subsequent edema and diminished central vision may be present. High-grade and atrophic papilledema in addition to subretinal hemorrhages are poor visual prognostic signs.
  • In some instances, the disc swelling is asymmetric, or, rarely, the appearance of the optic nerve may be relatively normal.
  • If left untreated, chronic disc swelling eventually leads to clinically significant visual loss. Although all patients present with enlarged blind spots during their initial perimetry, uncontrolled papilledema results in progressive peripheral visual field constriction or nerve fiber bundle defects (eg, nasal depression, nasal steps, arcuate scotomas).
  • The central visual field is affected in end-stage chronic papilledema.
  • Sudden loss of central vision may result from an associated anterior ischemic optic neuropathy, a vascular occlusion, or an associated subretinal neovascular membrane.
  • The diplopia noted in patients with idiopathic intracranial hypertension is invariably due to unilateral or bilateral sixth nerve palsy. These cranial nerve palsies diminish with the lowering of the intracranial pressure.
  • Occasionally, patients with diplopia present with oculomotor or trochlear nerve palsy.
  • In rare instances, vertical diplopia is due to a skew deviation.
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Causes

  • Most cases of idiopathic intracranial hypertension occur in young women who are obese and, less frequently, in men who are otherwise healthy. Patients with higher body mass indexes and recent weight gain are at an increased risk for this disorder.[5, 7]
  • If this disorder presents in an individual who is not overweight, ruling out associated risk factors is necessary. These risk factors include systemic diseases (including Lyme disease), disruption of cerebral venous flow, certain endocrine or metabolic disorders, and exposure to or withdrawal from certain exogenous substances.[3, 5]
  • Risk factors
    • Exogenous substances
      • The list of exogenous substances associated with idiopathic intracranial hypertension is extensive. Although an association exists between these substances and this disorder, the exact causal relationship is somewhat lacking in the literature.
      • Exogenous substances associated with idiopathic intracranial hypertension include amiodarone, antibiotics (eg, nalidixic acid, penicillin, tetracycline), carbidopa, levodopa, chlordecone, corticosteroids (eg, topical, systemic), cyclosporine, danazol, growth hormone, indomethacin, ketoprofen, lead, leuprolide acetate, levonorgestrel implants, lithium, oral contraceptives, oxytocin, perhexiline, phenytoin, and vitamin A (>100,000 U/d)/retinoic acid.[3, 5]
      • In some instances, although a patient may present with idiopathic intracranial hypertension following exposure to a certain medication, the disorder can continue despite the cessation of the presumed offending agent.
      • Withdrawal from corticosteroids may result in idiopathic intracranial hypertension.[3] If corticosteroids are used for the treatment of idiopathic intracranial hypertension, their withdrawal may lead to a rebound increase in intracranial pressure.[1]
    • Systemic diseases
      • A myriad of illnesses are associated with idiopathic intracranial hypertension. Some of these disorders result in an increased viscosity of the cerebrospinal fluid. However, in most of the listed entities, the causal link with raised intracranial pressure is not clear.
      • The following diseases have been associated with idiopathic intracranial hypertension: anemia,[14] chronic respiratory insufficiency, familial Mediterranean fever, hypertension, multiple sclerosis, polyangiitis overlap syndrome, psittacosis, renal disease, Reye syndrome, sarcoidosis, systemic lupus erythematosus, and thrombocytopenic purpura.[3]
    • Disorders of cerebral venous drainage
      • Cerebral venous compression by extravascular tumors, secondary thrombosis due to a coagulopathy, or relative stenosis due to a venous flow anomaly can result in impaired absorption of the cerebrospinal fluid and, thus, idiopathic intracranial hypertension.[4, 15] Restriction of venous drainage from the head may be impaired with radical neck dissection, even if completed only on the right side (predominant drainage from the head is via the right jugular vein). Spontaneous recanalization usually occurs, but, if delayed, chronic papilledema may result.
      • The diagnosis of cerebral sinus thrombosis may be missed with the exclusive use of CT. Therefore, either in patients who present atypically or in management dilemmas, ruling out cerebral venous thrombosis with the use of MRI/venography is worthwhile.
    • Endocrine disturbances: Pregnancy is occasionally associated with idiopathic intracranial hypertension.[1] This disorder can present at any stage of pregnancy. Given the limitations of neuroimaging studies and of medically treating patients who are pregnant, both the diagnosis and the management of these patients are determined on a case-by-case basis. Any neuroimaging studies or therapeutics should be performed in conjunction with the patient's obstetrician.
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Contributor Information and Disclosures
Author

Mark S Gans, MD  Associate Professor, Director of Neuro-Ophthalmology, Department of Ophthalmology, McGill University; Clinical Director, Department of Ophthalmology, Adult Sites, McGill University Hospital Center, Interim Chairman of the Department of Ophthalmology, McGill University

Mark S Gans, MD is a member of the following medical societies: American Academy of Ophthalmology, Canadian Medical Association, Canadian Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Edsel Ing, MD, FRCSC  Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Consulting Staff, Toronto East General Hospital, Canada

Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Ophthalmic Plastic and Reconstructive Surgery, Canadian Ophthalmological Society, North American Neuro-Ophthalmology Society, and Royal College of Physicians and Surgeons of Canada

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

Brian R Younge, MD  Professor of Ophthalmology, Mayo Clinic School of Medicine

Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society

Disclosure: Nothing to disclose.

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuroophthalmol. Jun 2004;24(2):138-45. [Medline].

  2. Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology. Nov 26 2002;59(10):1492-5. [Medline].

  3. Miller NR, Newman NJ. Pseudotumor cerebri (benign intracranial hypertension). In: Walsh and Hoyt's Clinical Neuro-Ophthalmology. Vol 1. 5th ed. 1999:523-38.

  4. 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].

  5. Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. Mar 2008;8(2):87-93. [Medline].

  6. Corbett JJ. The first Jacobson Lecture. Familial idiopathic intracranial hypertension. J Neuroophthalmol. Dec 2008;28(4):337-47. [Medline].

  7. 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].

  8. 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].

  9. 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].

  10. Ney JJ, Volpe NJ, Liu GT, Balcer LJ, Moster ML, Galetta SL. Functional Visual Loss in Idiopathic Intracranial Hypertension. Ophthalmology. Jul 28 2009;[Medline].

  11. Bruce BB, Kedar S, Van Stavern GP, et al. Idiopathic intracranial hypertension in men. Neurology. Jan 27 2009;72(4):304-9. [Medline].

  12. Jiraskova N, Rozsival P. Idiopathic intracranial hypertension in pediatric patients. Clin Ophthalmol. Dec 2008;2(4):723-6. [Medline].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. Brazis PW. Clinical review: the surgical treatment of idiopathic pseudotumour cerebri (idiopathic intracranial hypertension). Cephalalgia. Dec 2008;28(12):1361-73. [Medline].

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

  19. Sinclair AJ, Kuruvath S, Sen D, et al. Is cerebrospinal fluid shunting in idiopathic intracranial hypertension worthwhile? A 10-year review. Cephalalgia. Dec 2011;31(16):1627-33. [Medline].

 
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