Spongy degeneration of the central nervous system in infancy

Abstract

The clinical and pathological features of 7 cases of spongy degeneration of the nervous system have been described and those of an 8th case, originally reported by Canavan, have been reviewed. An analysis of these cases, as well as of 24 previously described cases derived from the medical literature, leaves little doubt that we are dealing with a clinicalpathological entity. The nature of this disease is not understood, but it probably should be classed with the familial degenerations of the white matter or leukodystrophies of infancy. In the present state of our knowledge, it seems suitable to designate this disease by its most distinctive pathological attribute, namely, spongy degeneration, as originally suggested by van Bogaert and Bertrand.