What Causes Idiopathic Intracranial Hypertension?

Patient Presentation
A 14-year-old female came to the emergency room with a 3 week history of tinnitus that resolved 3 days prior to admission.
She had persistent blurred vision in her left eye and 2 days ago developed it in her right eye. She also complains of some neck pain and a headache that she was taking ibuprofen for. She denied any trauma.
The past medical history showed attention deficit disorder on methylphenidate and mild acne that was controlled with benzoyl peroxide.
The family history was negative for any hematological, neurological or ophthalmological disorders.
The review of systems showed no fever, nausea, vomiting, cough, diarrhea, difficulties walking, writing or speaking.
The pertinent physical exam showed vital signs of blood pressure = 145/105, pulse = 98, respiratory rate = 20, was afebrile, with a mini-mental status evaluation showing her to be alert and oriented x 3.
Her weight was > 95% for age with a body mass index of 35 kg/m2. Eye examination showed decreased light perception bilaterally, with papilledema and her venous pulsations could not be determined. Extraocular muscles were intact.
Neurologically cranial nerves III-XII were intact, deep tendon reflexes were normal, strength and tone were normal. She had normal sensation and was able to walk but had problems because of her vision.
She had no obvious ataxia.
The work-up included an emergency consultation with ophthalmology and neurology that confirmed decreased vision, papilledema and no venous pulsations.
She was taken to the pediatric intensive care unit, given intravenous Diamox®, Lasix® and Solumedrol®.
Magnetic resonance imaging revealed the diagnosis of a non-occulsive venous sinus thrombosis in the right transverse sinus, but it was questioned whether or it this alone could be the cause of her idiopathic intracranial hypertension.
A lumbar puncture was normal.
The patient’s clinical course showed her to have a continued decrease in her vision over the next day and therefore a lumboperitoneal shunt was placed to decrease the intracranial pressure.
This stopped the visual decline but at discharge she had not regained her prior vision and had a discharge acuity of 20/150 in her right eye and 20/180 in her left eye.
The diuretics were stopped and she was to continue prednisone, tapering it over 1 month.
During her admission she was evaluated for her hypertension and her idiopathic hypertension was being controlled on propranolol at discharge.
She had a hematological evaluation for a hypercoagulable state that was negative, but would have repeated Protein C and Protein S levels drawn later as acute levels may not be accurate. She was placed on aspirin prophylaxis.
She was discharged to home with daily occupational therapy and physical therapy. She was to follow up with a local nutritionist for her obesity, with her local physician within the week and other specialists within the month.

Figure 63 – Axial (left) and sagittal (right) T1-weighted
magnetic resonance images of the brain demostrate a relatively small
bright (seen here as white) signal abnormality in the right
transverse sinus, concerning for venous sinus thrombosis.

Figure 64 – 06-30-08 – Magnetic resonance venogram obtained with
contrast demonstrates some irregularity in the contour of the right
transverse sinus, but it is still patent and there is flow within it,
demonstrating the thrombus is non-occlusive.

Papilledema is described as congestion and elevation of the optic nerve head, blurring of the optic nerve disc margin, obliteration of the physiological optic cup, venous congestion with loss of spontaneous venous pulses, peripapillary splinter hemorrhages and edema of the peripapillary retina.

An image of papilledema from EyeRounds.org can be seen by clicking here.
Papilledema is a neurological emergency and needs prompt evaluation by specialists.

The causes of papilledema include:

  • Cataracts
  • Demyelinating diseases
  • Drugs
  • Glaucoma
  • Hypertension
  • Iridocyclitis
  • Meningo-encelphalitis
  • Optic atrophy
  • Tumor
  • Retinitis pigmenotosa

Idiopathic intracranial hypertension (IIH) is the proper term but many other terms are used such as benign intracranial hypertension or pseudotumor cerebri which is the term used by the National Library of Medicine.
IIH is defined as elevated intracranial pressure but no clinical laboratory or radiographic evidence of hydrocephalus, infection, tumor or vascular abnormality.
IIH has papilledema, normal or small cerebral ventricular system on radiographic imaging, no intracranial mass and normal cerebrospinal fluid.
The pathogenesis of IIH is not complete understood. Proposed mechanisms include increased cerebral blood volume, increased cerebrospinal fluid secretion, increased venous sinus pressure and decreased cerebrospinal fluid absorption. All or combinations of these problems can lead to intracranial hypertension.

IIH occurs from childhood (with an increasing incidence in adolescence) to adulthood. Infants and neonates usually do not have it.
In younger children there is an equal gender distribution of patients, but as age increases female predominate.

Patients often come to attention because of headache, nausea and/or vomiting, blurred vision or diplopia, dizziness, tinnitus and stiff neck.
Other presentations include anorexia, increasing head size, pain behind the orbit, photophobia, head tilt and preference for knee-chest position.
Patients can also be asymptomatic with papilledema noticed incidentally on physical examination.
The papilledema in IIH is usually bilateral but may be unilateral or asymmetric.

Treatment to decrease the intracranial pressure and to maintain vision include stopping possible causative drugs, diuretics (i.e. acetazolamide, furosemide), serial lumbar punctures, and operative procedures such as optic nerve sheath fenestration or placement of a lumboperitoneal shunt.
With prompt treatment, visual loss can be completely reversible, but some visual loss is reported in up to 17% of patients. Recurrence rate is between 6-12%

Learning Point
Causes and possible associations with IIH include:

  • Cardiovascular
    • Heart murmur
    • Mitral valve prolapse
    • Heart transplant*
  • Collagen Vascular
    • Behçet disease
    • Panuveitis
    • Polyangiitis overlap syndrome
    • Systemic lupus erythematosus
  • Drugs
    • Amiodarone
    • Antibiotics – minocycline and tetracycline
    • Chemotherapy – cytarabine
    • Contraceptives, oral and implantable
    • Danazol
    • Desmopressin
    • Human growth hormone
    • Steroids or steroid withdrawal
    • Lithium
    • Mesalamine
    • Nalidixic acid
    • Nitrofurantoin
    • Vitamin A, retinoic acid and similar drugs
  • Endocrine
    • Addison disease
    • Cushing disease
    • Hypocalcemia
    • Hypo or hyperthyroidism
    • Ovarian dysfunction
    • Vitamin D-dependent rickets
    • Vitamin A excess or deficiency
  • Gastrointestinal
    • Crohn’s disease
  • Genetic
    • Guillain-Barré
    • Galactosemia
    • Miller-Fisher variant
  • Hematologic
    • Severe anemia
    • Hypercoagulable states*
    • Polycythemia vera
    • Wiskott-Aldrich syndrome
  • Infectious Disease
    • Illness with fever
    • Lyme disease*
    • Measles
    • Otitis media
    • Roseola
    • Sinusitis
    • Streptococcal B pharyngitis
    • Tonsillitis
    • Upper respiratory tract infection
    • Viral disease
    • Varicella
  • Pulmonary
    • Asthma
    • Chronic lung disease
    • Cystic fibrosis*
  • Renal
    • Renal failure*
    • Renal transplant*
  • Surgical/Trauma
    • Appendectomy
    • Head trauma*
    • Tonsillectomy and adenoidectomy
  • Miscellaneous
    • Malnutrition
    • Obesity
    • Postvaccination
    • Refeeding

Note: one paper (Rangwala and Liu, see To Learn More below) reviewed other reports which may not meet the standard criteria for IIH. This paper cites the following as possibly not meeting criteria for IIH: cystic fibrosis, head trauma, heart transplantation, hypercoagulable states, Lyme disease, renal failure and renal transplantation.

Questions for Further Discussion
1. What radiographic imaging tests should be performed to evaluate papilledema?
2. What disease processes are considered neurological emergencies?
3What disease processes are considered opthalmological emergencies?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Information prescriptions for patients can be found at MedlinePlus for this topic: <a href="Benign Tumors .

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Illingworth RS. Common Symptoms of Disease in Children. Blackwell Scientific Publications: Oxford. 1998:188.

Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:2398.

Mercille G, Ospina LH. Pediatric idiopathic intracranial hypertension: a review.
Pediatr Rev. 2007 Nov;28(11):e77-86.

Rangwala LM, Liu GT.
Pediatric idiopathic intracranial hypertension.
Surv Ophthalmol. 2007 Nov-Dec;52(6):597-617.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Interpersonal and Communication Skills
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Systems Based Practice
    23. Differing types of medical practice and delivery systems including methods of controlling health care costs and allocating resources are known.
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital

    June 30, 2008