An Example of Status Marmoratus of the Cerebral Cortex.

THE phenomenon of hypermyelinization has received much attention from neuropathologists since C. Vogt's (1911) original description of status marmoratus of the corpus striatum. It is now well established that hypermyelinized fibres in the form of plaques or as the peculiar networks which first suggested the name " marbled " may be present in many parts of the nervous system, though they are most common in the basal ganglia and cerebral cortex. A review of the recorded cases shows that several etiological factors may be operative in the production of this remarkable state (Hallervorden, 1930). Thus, hypermyelinized plaques have been found associated with traumatic lesions (Freund and Vogt, 1911; Pfeiffer, 1925; Scholz, 1924), general paralysis (Bielschowsky, 1924), disseminate sclerosis (Wertham, 1924), von Recklinghausen's disease (Bielschowsky, 1924), senile dementia (Stief, 1924), telangiectiasis of the cortex (Bielschowsky, 1924), carbon monoxide poisoning (Spatz, 1931), febrile illness in infancy (Anton, 1896; Scholz, 1924; Case, 1934 ; Lowenberg and Malamud, 1933), and birth injury (Meyer and Cook, 1936). Familial instances of status marmoratus of the corpus striatum are also on record (Anton, 1896; Scholz, 1924; Vogt, 1920). The pathogenesis of the condition is obscure. Since hypermyelinization is almost invariably accompanied by some degree of gliosis, there are grounds for believing that a perversion of glial function has led to the abnormal myelin production. Considerable uncertainty, however, still exists as to the mode of origin of the abundant axis cylinders found in the plaques. Shrinkage of the tissues might conceivably produce the linear hypermyelinized scars so frequently seen in the cerebral cortex, but it is difficult to imagine how such a mechanism could produce the distinctive networks of a true status marmoratus. Bielschowsky's (1924) suggestion that previously naked axis cylinders have become myelinized also fails to explain the excessive number and peculiar arrangement of the fibres. Most authors seem to have followed Scholz (1924) in interpreting the abnormal axis cylinder development as a regenerative phenomenon, despite the fact that in experimental animals only ephemeral and abortive regenerative activity has been observed. The experiments of Borst (1904) have been cited as proving that a new growth of myelinized fibres can actually occur after cerebral lesions, but, as Cajal (1928) has pointed out, his findings are open to other interpretations and he did not employ neurofibrillar staining methods. I have thought the following case worthy of record since, among other points of interest, it exemplifies the association of status marmoratus with certain cortical abnormalities of prenatal origin.