The authors retrospectively examined the performance of recently revised diagnostic criteria for pseudotumor cerebri syndrome.

Study design

The revised criteria requires three of four neuroimaging findings in the absence of papilledema for diagnosing pseudotumor cerebri syndrome. The cohort was comprised of children with definite or probable pseudotumor cerebri syndrome and those with elevated opening pressure but without papilledema. Control subjects had normal opening pressure without papilledema. The neuroradiologist reviewed MRI scans to evaluate for four signs: pituitary gland flattening/empty sella, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis.

Outcomes

Identification of three or more MRI scan findings had a sensitivity of 62% and specificity of 95%. Two of three signs (transverse venous sinus stenosis, pituitary gland flattening/empty sella, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity and specificity.

Limitations

This study was limited by its retrospective design and relatively small sample size (n=119).

Clinical significance

The authors conclude that in children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity, but a robust specificity, for pseudotumor cerebri syndrome. Clinicians should review MRI scans for these criteria, and should consider a diagnosis of pseudotumor cerebri syndrome if the suspicious signs are present, particularly if papilledema is uncertain or in question.

These data argue against the need for diagnostic evaluation for pseudotumor cerebri syndrome in children, unless papilledema, transverse venous sinus stenosis, or three of four imaging criteria are present—possibly avoiding the need for lumbar puncture to be performed.