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Idiopathic Intracranial Hypertension Workup

  • Author: Mark S Gans, MD; Chief Editor: Andrew G Lee, MD  more...
Updated: Jan 28, 2016

Laboratory Studies

Blood tests

Blood tests are useful for ruling out systemic lupus erythematosus or other collagen-vascular diseases, which have been reported as underlying conditions in some patients who present with idiopathic intracranial hypertension (IIH).[53]

An increased incidence of anticardiolipin antibodies has been reported in patients with IIH. Accordingly, some authors advocate anticardiolipin antibody assessment in all IIH patients, regardless of prior history of thrombosis.[54] Some authors advocate screening for anticardiolipin antibodies and other procoagulant states in all patients with IIH who are either male or nonobese.[55]

Cases of IIH associated with Lyme disease have been reported.[56]

Most patients with typical history, gender, and body habitus need only routine blood work, if any. Recommended blood tests include the following:

  • Complete blood count (CBC)
  • Erythrocyte sedimentation rate (ESR)
  • Serum iron and iron-binding capacity
  • Antinuclear antigen (ANA) profile (eg, anti-dsDNA and anti-ssDNA)
  • Full procoagulant profile, including protein S, protein C, homocysteine levels, antithrombin III, factor V Leiden variant, antiphospholipid/anticardiolipin antibodies, lupus anticoagulant, and platelet aggregation studies (in patients with a previous history of thrombosis or MRI evidence of dural venous sinus occlusion on magnetic resonance imaging [MRI])

Lyme screening test (enzyme-linked immunosorbent assay [ELISA]) in patients who have a history of exposure to Lyme in areas of endemic disease

CSF tests

CSF studies include the following:

  • Opening pressure
  • White blood cell (WBC) and differential counts
  • Red blood cell (RBC) count
  • Total protein
  • Quantitative protein electrophoresis
  • Glucose
  • Aerobic bacterial culture and sensitivity
  • Acid-fast bacilli (AFB) culture
  • Cryptococcal antigen (especially in patients with HIV)
  • Syphilis markers (eg, rapid plasma reagin [RPR])
  • Tumor markers and cytology (in patients with a history of cancer or with clinical features suggesting occult malignancy)

Most patients with typical history, gender, and body habitus need only routine CSF tests. However, extra fluid should be frozen in case the preliminary workup reveals unexpected abnormalities such as pleocytosis or elevated gamma globulin, which would indicate that more complete investigation for autoimmune, infectious, or neoplastic conditions is warranted.


MRI and CT

A patient with bilateral disc swelling should undergo urgent neuroimaging studies to rule out an intracranial mass or a dural sinus thrombosis. In the setting of IIH, the findings on neuroimaging studies include normal or small slitlike ventricles, enlarged optic nerve sheaths, and, occasionally, an empty sella.

MRI of the brain with gadolinium enhancement is probably the study of choice for all patients with IIH, in that it provides sensitive screening for hydrocephalus, intracerebral masses, meningeal infiltrative or inflammatory disease, and dural venous sinus thrombosis. In a retrospective study of imaging features that have been suggested as typical for patients with IIH, only flattening of the posterior globe was found to be a reliable indicator of IIH, with a specificity of 100% and a sensitivity of 43.5%.[57]

MR venography can be useful for patients who are at greater risk for dural venous sinus thrombosis, such as those with suspected thrombosis on MRI, nonobese or male individuals, or those with a documented procoagulant state. Sagittal T1-weighted images often provide excellent views of the superior sagittal sinus, and these typically are included in routine MRI. Extraluminal narrowing of the transverse sinuses may be a typical feature of IIH, as reported by Farb et al.[8]

Computed tomography (CT) of the brain is less expensive than MRI and is adequate to rule out an intracranial lesion in most instances; however, MRI and magnetic resonance (MR) venography are more effective in ruling out a mass lesion and a dural sinus thrombosis, respectively. Although MR venography was once considered an elective imaging study for atypical patients, it is now increasingly accepted as a routine study for all patients with IIH.[58]



Bedside ultrasonography has been used to identify intracranial hypertension by precisely measuring the diameter of the optic nerve sheath.[59] If this diameter increases in primary gaze and diminishes by 25% in eccentric gaze (30° test), then increased subarachnoid fluid surrounding the optic nerve is presumably present. This finding is consistent with papilledema if it is bilateral.

The drawback of this noninvasive technique is that it requires a highly skilled clinician to obtain reproducible results.


Lumbar Puncture

Once an intracranial mass lesion is ruled out, lumbar puncture is indicated. The opening pressure should be measured with the patient relaxed in the decubitus position to prevent a falsely elevated pressure reading. If any specific difficulty was encountered that may have caused such as false elevation, the clinician performing the procedure must communicate this to the ophthalmologist. Unfortunately, some patients demonstrate a transiently normal pressure despite harboring IIH; confirming the disease in these patients is difficult.

Besides the value of the opening pressure, the clarity and the color of the cerebrospinal fluid should be noted. In addition, the cerebrospinal fluid (CSF) should be forwarded for cell count, cytology, culture, and measurement of glucose, protein, and electrolyte concentrations. All of these findings are normal in patients with IIH.

In obese patients, finding landmarks may be difficult; consequently, the tap is often performed with the patient seated. It should be kept in mind that the normal CSF pressure at the foramen magnum in the seated position is nearly 500 mm water from the lumbar entry point in persons of average height. Thus, an opening pressure of 500 mm water is extremely high for the lateral decubitus position but normal for the sitting position. If possible, the patient should be moved to the lateral decubitus position before the pressure is measured.

Another approach to lumbar puncture in obese patients utilizes fluoroscopic guidance in the radiology department. The prone positioning on the x-ray table and the increased abdominal pressure in this position may elevate the CSF pressure falsely. If the pressure is normal with the patient in the prone position, then the measurement is probably accurate. However, if it is high, the patient must be rolled into the lateral decubitus position and allowed to relax before a reliable pressure reading can be completed.

Obviously, such maneuvers carry a risk of displacing the needle from the thecal space. However, no alternative method exists for obtaining an accurate pressure reading.

Contributor Information and Disclosures

Mark S Gans, MD Associate Professor, Director of Neuro-Ophthalmology, Interim Chair, Department of Ophthalmology, McGill University Faculty of Medicine; Clinical Director, Department of Ophthalmology, Adult Sites, McGill University Hospital Center, Canada

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

Disclosure: Nothing to disclose.

Chief Editor

Andrew G Lee, MD Chair, Department of Ophthalmology, Houston Methodist Hospital; Clinical Professor, Associate Program Director, Department of Ophthalmology and Visual Sciences, University of Texas Medical Branch School of Medicine; Clinical Professor, Department of Surgery, Division of Head and Neck Surgery, University of Texas MD Anderson Cancer Center; Professor of Ophthalmology, Neurology, and Neurological Surgery, Weill Medical College of Cornell University; Clinical Associate Professor, University of Buffalo, State University of New York School of Medicine

Andrew G Lee, MD is a member of the following medical societies: American Academy of Ophthalmology, Association of University Professors of Ophthalmology, American Geriatrics Society, Houston Neurological Society, Houston Ophthalmological Society, International Council of Ophthalmology, North American Neuro-Ophthalmology Society, Pan-American Association of Ophthalmology, Texas Ophthalmological Association

Disclosure: Received ownership interest from Credential Protection for other.


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.

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.

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.

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.

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Left optic disc with moderate chronic papilledema in a patient with idiopathic intracranial hypertension (pseudotumor cerebri). Paton lines (arc-shaped retinal wrinkles concentric with the disc margin) are seen along the temporal side of the optic nerve head.
Right optic disc with postpapilledema optic atrophy in a patient with idiopathic intracranial hypertension (pseudotumor cerebri). Diffuse pallor of disc and absence of small arterial vessels on surface are noted, with very little disc elevation. Disc margin at upper and lower poles and nasally is obscured by some residual edema in nerve fiber layer and gliosis that often persists even after all edema has resolved.
Most common early visual field defect in papilledema as optic nerve develops optic atrophy is inferior nasal defect, as shown in left eye field chart (left side of figure). Shaded area indicates defective portion of field. Note sharp line of demarcation between defective lower nasal quadrant and normal upper nasal quadrant along horizontal midline. This is characteristic of early papilledema optic atrophy and is referred to as nasal step or inferonasal step.
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