Status Marmoratus

Abstract

This syndrome was first established by C. and O. Vogt, 1 who considered as its most characteristic feature the occurrence of quantitatively and qualitatively abnormal myelinated fibers in the striate body, giving it a peculiar marble-like appearance. They considered this condition as restricted to the striate body, and advanced the hypothesis that it was the result of a faulty development. Subsequent findings showed that the original assumptions were not justified. Bielschowsky 2 found similar changes in the cortex, showing that the condition was not restricted to the striate body; Anton 3 , Meyer 4 , Scholz 5 , and others suggested that the lesion might be the result of some acquired process (inflammatory or other vascular) rather than a developmental deviation.

Our investigations were particularly concerned with these 2 phases of the problem, and are based on a study of 4 cases. Histological examination of 3 of these left no doubt that the condition was that of “Status Marmoratus”, whereas the fourth case, still living, shows a clinical development and picture that makes this diagnosis quite probable. In all of these there was no hereditary predisposition to this disease, and the early development was such that in at least 3 of the 4 there was no reason to suppose that they were not normal to begin with. In all of these the condition seems to have developed following acquired diseases. Histologically too the condition appears to have developed on the basis of acquired processes, most probably inflammatory. In one of the cases, the “status marmoratus,” furthermore, was limited to the cortex, none of the lesions being found in the striate body. In this case the condition is chiefly localized around blood vessels which show signs of a previous inflammatory process.