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Mucopolysaccharidosis I and intracranial tumor in a patient with high-pressure hydrocephalus

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Abstract

In a 19-month-old patient with mucopolysaccharidosis I (Pfaundler-Hurler, MPS I/H) high-pressure hydrocephalus required the implantation of a ventriculoperitoneal shunt. Despite a reduction in both ventricular volume and intracranial pressure, clinical symptoms suggesting compression of the brain stem persisted. Brain MRI revealed a tumor within the posterior cranial fossa. Cytologic examination of the cerebrospinal fluid was suggestive of a poorly differentiated ependymoma. Highpressure hydrocephalus is a common complication in MPS I/H. As changes in mucopolysaccharide metabolism may be associated with an increased risk of developing neoplasms, the possibility of an intracranial tumor should be considered in patients with MPS I/H and high-pressure hydrocephalus.

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Authors and Affiliations

  1. Department of Pediatrics, University of Graz, Auenbruggerplatz 30, A-8036, Graz, Austria

    S. Stöckler & E. Paschke

  2. Department of Pathology, University of Graz, Graz, Austria

    R. Kleinert

  3. Department of Radiology, University of Graz, Graz, Austria

    F. Ebner

Authors
  1. S. Stöckler
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  2. R. Kleinert
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  3. F. Ebner
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  4. E. Paschke
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Stöckler, S., Kleinert, R., Ebner, F. et al. Mucopolysaccharidosis I and intracranial tumor in a patient with high-pressure hydrocephalus. Pediatr Radiol 23, 353–354 (1993). https://doi.org/10.1007/BF02011955

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  • DOI: https://doi.org/10.1007/BF02011955

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