Neuropediatrics 1988; 19(4): 197-200
DOI: 10.1055/s-2008-1052445
Original articles

© Georg Thieme Verlag KG Stuttgart · New York

Gliomatosis Cerebri in a Newborn

P. G. Barth1 , F. C. Stam2 , W.  Hack3 , H. A. Delemarre-van de Waal4
  • 1Department of Neuropathology, Free University Hospital, Amsterdam, The Netherlands
  • 2Department of Neuropathology, Free University Hospital, Amsterdam, The Netherlands
  • 3Department of Pediatrics, Free University Hospital, Amsterdam, The Netherlands
  • 4Department of Pediatrics, Free University Hospital, Amsterdam, The Netherlands
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Publikationsverlauf

Publikationsdatum:
19. März 2008 (online)

Abstract

A newborn is reported with diffuse gliomatosis involving the cerebral hemispheres, the brainstem and the cerebellum.

The presenting signs were paucity of spontaneous movements except for multifocal clonic seizures, absent response to sensory stimuli and optic atrophy. A CT scan suggested agyria. The child expired on the seventh day. Autopsy disclosed diffuse gliomatosis affecting both cerebral hemispheres, the brainstem and the whole cerebellum, but excluding the spinal cord. Neuronal loss was unusually severe in all the affected areas. The genitals were ambiguous, an association not explained by the cerebral pathology. The karyotype was 46XY (male pseudohermaphroditism).

This is probably the first reported instance of gliomatosis cerebri in a newborn.