Clinical Consequences of the Revised Diagnostic Criteria for the Diagnosis of Idiopathic Intracranial Hypertension

Background: The criteria for the diagnosis of idiopathic intracranial hypertension (IIH) were revised in recent years.¹

Objective: Do the modified criteria of IIH change the diagnosis in clinical practice?

Methods: In a retrospective cross-sectional study, we found 20 children with the diagnosis of pseudotumor cerebri in the database of the department of Pediatric Neurology of the University Children’s Hospital Basel between January 1, 2006 and December 31, 2013. One case was excluded because of incomplete documentation. The diagnoses were reevaluated on the basis of the revised criteria mentioned earlier.

Results: All 19 remaining cases had lumbar puncture opening pressures above 28 cm H₂O. In four cases, the diagnosis was revised because of an assumed cause of intracranial pressure increase (secondary intracranial hypertension: Graves disease, venous sinus thrombosis, chronic subdural hematoma, and treatment with tacrolimus). In five children, neither papilledema nor a sixth nerve palsy was present. None of these patients satisfied the additional neuroimaging criteria (empty sella, flattening of the posterior aspect of the globe, distension of the perioptic subarachnoid space with or without a tortuous optic nerve, and transverse venous sinus stenosis). Therefore, 10 of the 19 originally classified as pseudotumor cerebri patients met the revised criteria of the ICH.

Conclusion: Because of discontinuation of headache and other ophthalmologic findings other than papilledema and sixth nerve palsy (e.g., visual field defects and enlarged blind spot) the diagnostic criteria are limited by the revised criteria, even if a significantly elevated lumbar puncture opening pressure is present.

Reference

Referenzen

1 Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81(13):1159–1165