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

BACKGROUND: Cerebrospinal fluid (CSF) opening pressure (OP) of ≥28 cm H2 O 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 H2 O if other diagnostic criteria are met. We report a group of children with probable PTCS.

METHODS: Retrospective analysis of 25 children diagnosed with PTCS but with a CSF OP below 28 cm H2 O. 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.

RESULTS: 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 >30 cm H2 O in seven children. MRI findings supportive of PTCS were seen in eight patients.

CONCLUSIONS: The diagnosis of probable PTCS as a subgroup of PTCS can be convincingly made in children with an OP < 28 cm H2 O. 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.