Revised Diagnostic Criteria for Pseudotumor Cerebri Syndrome in Adults and Children

September 25, 2013

Revised Diagnostic Criteria for Pseudotumor Cerebri Syndrome in Adults and Children

  1. Amy Gelfand, MD

An update from 2002 provides greater clarity.

  1. Amy Gelfand, MD

This article offers criteria for diagnosing pseudotumor cerebri syndrome in patients ranging from ages 3 to 60 years old, revised from criteria created in 2002. The authors advocate for using the inclusive diagnostic term “pseudotumor cerebri syndrome” rather than “benign intracranial hypertension” or “idiopathic intracranial hypertension,” as this vision-threatening condition is by no means benign and, in some patients, the precipitating cause is known rather than idiopathic. The syndrome can be subdivided into primary (i.e., idiopathic) and secondary forms. Secondary causes range from medication-induced to cerebral venous sinus thrombosis.

The authors offer diagnostic criteria for three clinical scenarios:

  • Pseudotumor cerebri syndrome with papilledema: Neurological examination is normal except for cranial nerve abnormalities; neuroimaging is normal except for findings suggestive of high pressure; cerebrospinal fluid (CSF) composition is normal; and the opening pressure of a properly performed lumbar puncture is elevated (≥250 mm CSF in adults and unsedated children and ≥280 mm CSF in sedated children).

  • Pseudotumor cerebri syndrome without papilledema: Criteria are the same as above, along with unilateral or bilateral sixth nerve palsies.

  • Suggested pseudotumor cerebri syndrome: Criteria for this scenario are fulfilled if there is no papilledema or sixth nerve palsy, but the other criteria are met and neuroimaging findings suggest high pressure.


These diagnostic criteria bring much needed clarity and organization to this challenging clinical area. If the nomenclature “pseudotumor cerebri syndrome” could be adopted consistently by clinicians, this alone would be useful. As the authors note, having an elevated CSF opening pressure in the absence of symptoms or signs suggestive of high pressure does not constitute a diagnosis of pseudotumor cerebri syndrome. Notably, headache is not part of the diagnostic criteria. Moreover, chronic daily headache is not, in itself, an indication for lumbar puncture to investigate for elevated pressure.

Dr. Gelfand is Assistant Professor, Department of Neurology, Division of Child Neurology, University of California, San Francisco.


Reader Comments (1)

Fadhil Abbas , FRCPI ,FRCP Physician, Neurology, Dubai , UAE

I would be grateful if the authors can let me know of the references for CSF pressure being elevated in the stated figures , invariably , and always in the pediatric age groups , and any comments they have on management implications of such adoption of their criteria in the group with no papilledema . Would a lesser figure than the stated make the responsible clinician , less confident in recommending the appropriate management in the second category .

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