Article Text

Download PDFPDF

Infantile spongy degeneration of the central nervous system associated with glycogen storage and markedly fatty liver
  1. Y. Takei,
  2. G. B. Solitare
  1. Department of Pathology (Neuropathology) Yale University School of Medicine, New Haven, Conn., U.S.A.


    The clinical, biochemical, and pathological features of an unusual expression of infantile spongy degeneration of the central white matter are presented with emphasis on neuropathological observations. The topographical distribution of the spongy change along with the observed defect in myelination were such as to suggest an arrest in development of the white matter in late foetal life. Of additional interest, in the present case, is the observed deposition of glycogen in the brain, heart, and liver along with a markedly fatty liver. Our findings are compared with those in other cases of so-called spongy degeneration, as well as with certain of the aminoacidurias and, as a consequence, we wish to suggest that the basic pathogenetic factor probably lies in a disturbance of the biochemical energy supply system rather than in a disorder of myelin lipid metabolism per se.

    Statistics from


    • 1 Supported in part by NINDS grant NSO6882 of the United Public Health Service.

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.