ARTÍCULOS

Revised Diagnostic Criteria for Pseudotumor Cerebri Syndrome in Adults and Children

An update from 2002 provides greater clarity.

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.
COMMENT

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.

Friedman DI et al. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013 Aug 21

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The International Classification of Headache Disorders,

3rd Edition (ICHD III) – Changes and Challenges 

Morris Levin, MD

Headache. 2013 Sep;53(8):1383-95

1. Ophthalmoplegic migraine has been renamed “Recurrent painful ophthalmoplegic neuropathy.”

2. "Retinal migraine requires symptoms referable to 1 eye, such as unilateral ocular vision loss or scotomata. But this subtype is exceedingly rare, and many question its existence as a migraine subtype.14 Most patients, of course, mistakenly report homonymous hemianopic visual changes as monocular, which indeed may be hard to differentiate."

3. Another change is the term change from Basilar-type migraine to “Migraine with Brainstem Aura.” These patients should sup- posedly have symptoms and/or signs suggestive of the posterior cerebral circulation such as bilateral visual symptoms, dysarthria, vertigo, hearing loss, DIPLOPIA, or ataxia, but the new label may be another misnomer as the evidence for brainstem dysfunction in migraine is scant. This category also requires at least 2 “brainstem symptoms” therefore excluding patients with typical migraine accompanied only by vertigo.