Child's Nervous System

, Volume 26, Issue 3, pp 313–321 | Cite as

Clinical spectrum of the pseudotumor cerebri complex in children

  • Daniel Tibussek
  • Dominik T. Schneider
  • Nicola Vandemeulebroecke
  • Bernd Turowski
  • Martina Messing-Juenger
  • Peter H. G. M. Willems
  • Ertan Mayatepek
  • Felix Distelmaier
Original Paper

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.

Keywords

Idiopathic intracranial hypertension Papilledema Secondary causes Acetazolamide Lumbar puncture Cerebrospinal fluid Visual field Ophthalmology 

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Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Daniel Tibussek
    • 1
  • Dominik T. Schneider
    • 2
  • Nicola Vandemeulebroecke
    • 3
  • Bernd Turowski
    • 4
  • Martina Messing-Juenger
    • 5
  • Peter H. G. M. Willems
    • 6
  • Ertan Mayatepek
    • 1
  • Felix Distelmaier
    • 1
  1. 1.Department of General Pediatrics, University Children’s HospitalHeinrich-Heine-University DüsseldorfDüsseldorfGermany
  2. 2.Clinic of PediatricsKlinikum DortmundDortmundGermany
  3. 3.Department of OphthalmologyHeinrich-Heine-UniversityDüsseldorfGermany
  4. 4.Department of Diagnostic RadiologyHeinrich-Heine-UniversityDüsseldorfGermany
  5. 5.Department of Neurosurgery, Asklepios Childrens Hospital, Sankt AugustinUniversity of BonnBonnGermany
  6. 6.Department of BiochemistryRadboud University Nijmegen Medical CenterNijmegenThe Netherlands

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