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Pseudotumor Cerebri: Differential Diagnoses & Workup

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

Differential Diagnoses

Arteriovenous Malformations
Lyme Disease
Aseptic Meningitis
Meningioma
Blood Dyscrasias and Stroke
Migraine Headache
Extraocular Muscles, Actions
Migraine Variants
Hydrocephalus
Pseudotumor Cerebri
Intracranial Epidural Abscess
Pseudotumor Cerebri: Pediatric Perspective
Intracranial Hemorrhage
Subarachnoid Hemorrhage
Leptomeningeal Carcinomatosis
Systemic Lupus Erythematosus
Low-Grade Astrocytoma
Lumbar Puncture (CSF Examination)

Other Problems to Be Considered

Abducens (CN VI) nerve palsy
Vascular malformations and hematomas of the brain

Workup

Laboratory Studies

  • Blood tests
    • Reasons for blood tests
      • Rule out systemic lupus erythematosus or other collagen-vascular disease, since these have been reported as underlying conditions in some patients who present with idiopathic intracranial hypertension (IIH).27
      • An increased incidence of anti-cardiolipin antibodies has been reported in patients with IIH. Some authors advocate anti-cardiolipin antibody assessment in IIH patients regardless of prior history of thrombosis.28 Some authors advocate screening for anti-cardiolipin antibodies and other procoagulant states in all patients with IIH who are either male or nonobese.29
      • Cases of IIH associated with Lyme disease have been reported.30
      • Most patients with typical history, gender, and body habitus need only routine blood work, if any.
    • Blood tests recommended
      • Complete blood count
      • Erythrocyte sedimentation rate
      • Serum iron and iron binding capacity
      • Anti-cardiolipin antibodies/lupus anticoagulant
      • Antinuclear antigen (ANA) profile (eg, anti-dsDNA, anti-ssDNA)
      • Full procoagulant profile including protein S, protein C, homocysteine levels, antithrombin III, factor V Leiden variant, anti-phospholipid/anti-cardiolipin antibodies, lupus anticoagulant, and platelet aggregation studies (in patients with previous history of thrombosis or MRI evidence of dural venous sinus occlusion)
      • Lyme screening test (enzyme-linked immunosorbent assay [ELISA]) in patients who have a history of exposure to Lyme in areas of endemic disease
  • CSF studies
    • Opening pressure
    • White blood cell and differential counts
    • Red blood cell 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, indicating that more complete investigation for autoimmune, infectious, or neoplastic conditions is warranted.

Imaging Studies

  • MRI: Brain MRI with gadolinium enhancement is probably the study of choice for all patients with IIH since 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 statistically to be a reliable indicator of IIH, with a specificity of 100% and a sensitivity of 43.5%.31
  • MR venography: 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 and coworkers.1
  • CT scan: Brain CT scan is less expensive than MRI and is adequate to rule out larger tumors or lesions, but it is not as sensitive as MRI for meningeal infiltration and/or dural venous sinus thrombosis.

Procedures

  • Lumbar puncture
    • Lumbar puncture ideally is carried out with the patient in the lateral decubitus position. Because finding landmarks is difficult in obese patients, the tap frequently is performed with the patient seated.
    • Remember 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.
    • Therefore, note that an opening pressure of 500 mm water is extremely high in the lateral decubitus position, but is normal for the sitting position. If possible, the patient should be moved to lateral decubitus position before measuring the pressure.
    • 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 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.

More on Pseudotumor Cerebri

Overview: Pseudotumor Cerebri
Differential Diagnoses & Workup: Pseudotumor Cerebri
Treatment & Medication: Pseudotumor Cerebri
Follow-up: Pseudotumor Cerebri
Multimedia: Pseudotumor Cerebri
References
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References

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Further Reading

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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

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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.

 
 
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