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October 9, 1967

Spongy Degeneration of the Brain in Infancy

Author Affiliations

Chapel Hill, NC

JAMA. 1967;202(2):157. doi:10.1001/jama.1967.03130150125045

This article is only available in the PDF format. Download the PDF to view the article, as well as its associated figures and tables.


This monograph, a translation from the French, is a comprehensive and critical review of the clinical, pathological, and genetic features of a rare, often familial disease. The authors have much to contribute, since they were instrumental in defining spongy degeneration of the brain in infancy as a distinct nosological entity. The initial chapters trace the steps in recognition of this as a new disease and include much of the authors' own material on their early cases, some of it not previously published. In a separate chapter, the cases from the literature are presented in detail and critically reviewed. The problem of differential diagnosis is well treated.

Although only one of the chapters is entitled "Histopathology," pathological descriptions appear throughout the book. Gross and microscopic pathology is fully described and aptly illustrated, though the quality of the reproductions is merely adequate.

In considering the pathogenesis and the relationship of this entity