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

Abstract

Cerebrospinal fluid (CSF) opening pressure (OP) of ≥28cm 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<28cm H2O if other diagnostic criteria are met. We report a group of children with probable PTCS. Retrospective analysis of 25 children diagnosed with PTCS but with a CSF OP below 28cm 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. Of 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 >30cm H2O in seven children. MRI findings supportive of PTCS were seen in eight patients. The diagnosis of probable PTCS as a subgroup of PTCS can be convincingly made in children with an OP<28cm 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.