Revised Diagnostic Criteria for Pseudotumor Cerebri Syndrome in Adults and Children

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.