Neuropediatrics 2006; 37(4): 253-256
DOI: 10.1055/s-2006-924576
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Georg Thieme Verlag KG Stuttgart · New York

Rapidly Progressive Vanishing White Matter Disease in a Child with Previously Inconspicuous Brain MRI

X.-Q. Ding1 , M. Goerg2 , B. Eckert3 , A. Ohlenbusch4 , A. Kohlschuetter2 , J. Gaertner4 , H. Zeumer1
  • 1Department of Neuroradiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
  • 2Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
  • 3Department of Neuroradiology, General Hospital Altona, Hamburg, Germany
  • 4Department of Pediatrics and Neuropediatrics, Georg-August-University, Goettingen, Germany
Further Information

Publication History

Received: December 31, 2005

Accepted after Revision: August 9, 2006

Publication Date:
20 December 2006 (online)

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Abstract

In this pediatric case of vanishing white matter disease with early onset, rapidly progressive course, and fatal outcome, the white matter vanishing process in patient was for the first time documented morphologically in detail: An initial magnetic resonance imaging documented a normal appearing brain maturation. Rapid progressive brain lesions initiated morphologically de novo in the former well myelinated deep white matter were observed six months later after disease onset, including concentric ongoing signs of restricted proton diffusion cytotoxic edema on diffusion weighted imaging. Cyst-like defects at the lesion center of the deep white matter were detected more clearly on MRI ten months later. A pathomechanism like tumor necrosis factor induced oligodendrocyte apoptosis and primary demyelination was postulated. The case demonstrates that in the presence of clinically progressive symptoms, the development of VWM is possible even if first MRI findings are negative.

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