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Neuropediatrics 1989; 20(2): 79-81
DOI: 10.1055/s-2008-1071269
Original article

© Georg Thieme Verlag KG Stuttgart · New York

Spongy Degeneration of the Neuraxis (Canavan-Van Bogaert Disease) and N-Acetylaspartic Aciduria

B.  Echenne1 , P.  Divry2 , C.  Vianey-Liaud2
  • 1Unité de Neuropédiatrie, Centre Gui de Chauliac, F-34059 Montpellier Cédex, France
  • 2Laboratoire de Biochimie, Hôpital Debrousse, F-69322 Lyon Cédex 05, France
Further Information

Publication History

Publication Date:
19 March 2008 (online)

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Abstract

Urinary excretion of an abnormal amount of N-acetylaspartic acid has been evident in a 17-month-old child with an infantile picture of Canavan-van Bogaert disease. This observation makes it possible to consider the possibility of a simple biological diagnosis and confirms the existence of metabolic abnormalities which will probably permit to make rapid progress in the physiopathologic study of this disease.

Key words

Canavan-van Bogaert disease - Myelin diseases - CT scan

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