Multicystic encephalopathy--a polyetiologic condition in early infancy: morphologic, pathogenetic and clinical aspects.

Eleven observations of severe multicystic encephalopathy ( MCE ) in young infants and in a two-year-old child provide the basis for a summing-up and discussion of the various aspects of this characteristic polyetiologic phenomenon occurring in early infancy. In all cases the triggering causes or underlying disorders were different, although in five cases the common pathogenetic mechanism was a disturbance of circulation and/or respiration (acute respiratory distress syndrome). In two cases the basic disorders were a suppurative and a granulomatous meningoencephalitis. Carbon monoxide poisoning had occurred in one and diffuse meningocerebral angiomatosis in another two cases. In the eleventh case, one of a complicated twin birth, the exact cause of the MCE remained obscure. These cases together with those recorded in the literature demonstrate that the surprisingly constant pattern of damage in MCE , which results from different etiologic conditions, should be due to a specific mode of reaction of the infantile brain to a common pathogenetic mechanism. Anoxia with hypercapnia and the formation of brain edema are discussed as the basic events in the pathogenesis of MCE .