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Neuropediatrics 1985; 16(4): 185-190
DOI: 10.1055/s-2008-1059534
ORIGINAL ARTICLES

© Georg Thieme Verlag KG Stuttgart · New York

A Neurophysiological Study of Children with the Cerebro-Hepato-Renal Syndrome of Zellweger

Lutgarde  Govaerts1 , E.  Colon2 , J.  Rotteveel1 , L.  Monnens1
  • 1Institute of Pediatrics, Sint Radboudhospital, Geert Grooteplein Zuid 20, NL-6500 HB Nijmegen, The Netherlands
  • 2Institute of Neurology, Department of Clinical Neurophysiology, Sint Radboudhospital, Renier Postlaan 4, NL-6500 HB Nijmegen, The Netherlands
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Publikationsdatum:
30. April 2008 (online)

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Abstract

The aim of this study was to describe the EEGs, brainstem auditory evoked potentials and somatosensory evoked potentials obtained in eleven children with the Zellweger syndrome. In six out of the eleven patients BAEPs and in five of them SSEPs were performed. Severe abnormalities could be demonstrated, reflecting diffuse cerebral dysfunction as well as long myelinated fiber tracts dysfunction. In four of six patients no response was obtained by BAEPs and in two the central conduction time was delayed. The specific (until 60 ms) and aspecific (after 60 ms) complex of the SSEPs was delayed or the potential could not be elicited.
During sleep as well as wakefulness characteristic abnormalities of the EEGs were found in nine patients. These abnormalities consisted of continuous negative sharp waves and spikes at the vertex.

Key words

Cerebro-hepato-renal syndrome of Zellweger (CHR-S) - Evoked potentials (EP) - EEG