Original articleProbable pseudotumor cerebri complex in 25 children. Further support of a concept

•CSF opening pressure ≥28 cm H2O is a diagnostic criterion of Pseudotumor cerebri syndrome (PTCS).•“Probable PTCS” can be diagnosed with an opening pressure <28 cm H2O when other diagnostic criteria are fulfilled.•“Probable PTCS” is a clinically relevant subgroup of pediatric PTCS.

BackgroundCerebrospinal fluid (CSF) opening pressure (OP) of ≥28 cm H2O is now considered a diagnostic criterion for Pseudotumor cerebri syndrome (PTCS) in children. However, it has been proposed that a diagnosis of “probable” PTCS can be made with an OP < 28 cm H2O if other diagnostic criteria are met. We report a group of children with probable PTCS.MethodsRetrospective analysis of 25 children diagnosed with PTCS but with a CSF OP below 28 cm H2O. Eleven patients were identified during a nation-wide, prospective, active hospital-based surveillance, and additional 14 patients from our own institution. An extensive chart review of these cases was performed in order to identify signs and symptoms supportive of PTCS.ResultsOf these 25 patients 23 were treated with acetazolamide. Five children required escalation of medical treatment. Findings supportive of PTCS in the absence of an abnormal OP were: papilledema (n = 24), abducens nerve palsy (n = 7), without papilledema in one of them, headache (n = 15). Six patients had a relapse. A second lumbar puncture (LP) documented an opening pressure of >30 cm H2O in seven children. MRI findings supportive of PTCS were seen in eight patients.ConclusionsThe diagnosis of probable PTCS as a subgroup of PTCS can be convincingly made in children with an OP < 28 cm H2O. Results of opening pressure measurement always need to be interpreted within the whole clinical context. Treatment decisions in patients with “probable” PTCS should follow the same stage-based principles as for “proven” PTCS.