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Clinical spectrum of the pseudotumor cerebri complex in children

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Abstract

Purpose

Our aim was to improve diagnosis and management of pseudotumor cerebri (PTC; also known as idiopathic intracranial hypertension) in children.

Methods

We performed a comprehensive analysis of epidemiology, diagnostic work-up, therapy, and clinical follow-up in 53 consecutive patients.

Results

We identified several important aspects to be considered in the management of these children. First, patients may present without obvious symptoms at diagnosis. Second, bilateral papilledema might not or not yet be present in symptomatic patients. Third, measurement of cerebrospinal fluid (CSF) opening pressure may not always be reliable due to drugs used for sedation, which may alter intracranial pressure. Fourth, normal CSF pressure values in childhood are not well established and diagnosis might even be justified if pressure is <20 cm H2O. Fifth, associated conditions are frequent (at least in our cohort); however, in most cases, a causative link cannot be proven. Finally, disease relapse is a serious problem (20% in our group), which stresses the importance of standardized follow-up programs.

Conclusions

PTC constitutes an important and possibly underrecognized disorder in children and adolescents. Considering the high percentage of possibly associated conditions in our study, a detailed diagnostic work-up is crucial to identify treatable underlying conditions.

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The authors report no conflicts of interest.

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Authors and Affiliations

  1. Department of General Pediatrics, University Children’s Hospital, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Germany

    Daniel Tibussek, Ertan Mayatepek & Felix Distelmaier

  2. Clinic of Pediatrics, Klinikum Dortmund, Dortmund, Germany

    Dominik T. Schneider

  3. Department of Ophthalmology, Heinrich-Heine-University, Düsseldorf, Germany

    Nicola Vandemeulebroecke

  4. Department of Diagnostic Radiology, Heinrich-Heine-University, Düsseldorf, Germany

    Bernd Turowski

  5. Department of Neurosurgery, Asklepios Childrens Hospital, Sankt Augustin, University of Bonn, Bonn, Germany

    Martina Messing-Juenger

  6. Department of Biochemistry, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

    Peter H. G. M. Willems

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  1. Daniel Tibussek
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  2. Dominik T. Schneider
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  3. Nicola Vandemeulebroecke
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  4. Bernd Turowski
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  5. Martina Messing-Juenger
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  6. Peter H. G. M. Willems
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  7. Ertan Mayatepek
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  8. Felix Distelmaier
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Correspondence to Felix Distelmaier.

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Tibussek, D., Schneider, D.T., Vandemeulebroecke, N. et al. Clinical spectrum of the pseudotumor cerebri complex in children. Childs Nerv Syst 26, 313–321 (2010). https://doi.org/10.1007/s00381-009-1018-0

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  • DOI: https://doi.org/10.1007/s00381-009-1018-0

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