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Gewählte Publikation:

Stöckler, S; Kleinert, R; Ebner, F; Paschke, E.
Mucopolysaccharidosis I and intracranial tumor in a patient with high-pressure hydrocephalus.
Pediatr Radiol. 1993; 23(5):353-354 Doi: 10.1007/BF02011955 (- Case Report)
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Co-Autor*innen der Med Uni Graz
Ebner Franz
Kleinert Reinhold
Paschke Eduard
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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 ventriculo-peritoneal 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. High-pressure 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.
Find related publications in this database (using NLM MeSH Indexing)
Brain Neoplasms - complications
Cranial Fossa, Posterior - complications
Ependymoma - complications
Female - complications
Humans - complications
Hydrocephalus - complications
Infant - complications
Mucopolysaccharidosis I - complications

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