Daniel Tibussek1, Felix Distelmaier2, Michael Karenfort3, Stefani Harmsen4, Dirk Klee5, Ertan Mayatepek6. 1. Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich-Heine-University, Moorenstrasse 5, 40225 Düsseldorf, Germany. Electronic address: daniel.tibussek@gmx.net. 2. Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich-Heine-University, Moorenstrasse 5, 40225 Düsseldorf, Germany. Electronic address: felix.distelmaier@med.uni-duesseldorf.de. 3. Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich-Heine-University, Moorenstrasse 5, 40225 Düsseldorf, Germany. Electronic address: michael.karenfort@med.uni-duesseldorf.de. 4. Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich-Heine-University, Moorenstrasse 5, 40225 Düsseldorf, Germany. 5. Department of Diagnostic and Interventional Radiology, Medical Faculty, University Dusseldorf, Moorenstrasse 5, 40225 Düsseldorf, Germany. Electronic address: dirk.klee@med.uni-duesseldorf.de. 6. Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich-Heine-University, Moorenstrasse 5, 40225 Düsseldorf, Germany. Electronic address: mayatepek@med.uni-duesseldorf.de.
Abstract
BACKGROUND: Cerebrospinal 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. METHODS: Retrospective 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. 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 H2O 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 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.
BACKGROUND: Cerebrospinal 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. METHODS: Retrospective 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. 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 H2O 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 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.
Authors: Ali G Hamedani; Kailyn F R Witonsky; Mahgenn Cosico; Robert Rennie; Riu Xiao; Claire A Sheldon; Grace L Paley; Shana E McCormack; Geraldine W Liu; Deborah I Friedman; Grant T Liu; Christina L Szperka Journal: Headache Date: 2018-08-23 Impact factor: 5.887