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Neuropediatrics 1984; 15(1): 43-48
DOI: 10.1055/s-2008-1052339
CASE REPORTS

© Georg Thieme Verlag KG Stuttgart · New York

The Wiedemann-Rautenstrauch or Neonatal Progeroid Syndrome - Neuropathological Study of a Case

J. J. Martin1 , C. M. Ceuterick1 , J. G. Leroy2 , E. A. Devos3 , J. G. Roelens3
  • 1Division of Neuropathology, Born-Bunge Foundation and Antwerp University Medical School, Antwerpen, Belgium
  • 2Division of Medical Genetics, Antwerp University Medical School, Antwerpen, Belgium
  • 3Division of Pediatrics and Division of Pathology, "Heilig Hart Hospitaal", Roeselaere, Belgium.
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Publication History

Publication Date:
14 May 2008 (online)

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Abstract

Neuropathological data from the autopsy of a 5œ year-old female patient with the Wiedemann-Rautenstrauch or neonatal progeroid syndrome are reported.

Extensive demyelination is found in the central nervous system with occasionally a tigroid pattern and with large amounts of neutral fats and intermediate debris of myelin breakdown in macrophages. These lesions are characteristic of pure sudanophilic leucodystrophy.

Features differentiating the findings from those in Pelizaeus-Merzbacher disease and in other disorders associated with sudanophilic leucodystrophy with various special characteristics are presented and discussed.

Key words

Wiedemann-Rautenstrauch syndrome - Neonatal progeroid syndrome - Sudanophilic leucodystrophy - Demyelination - Neutral fats

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