Multicystic encephalopathy--a polyetiologic condition in early infancy: morphologic, pathogenetic and clinical aspects
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Severe multicystic encephalopathy (MCE) in infants can stem from various causes, but often involves circulatory or respiratory issues. The infantile brain may react similarly to different triggers, leading to consistent damage patterns.
Area of Science:
- Neurology
- Pediatrics
- Pathology
Background:
- Severe multicystic encephalopathy (MCE) is a condition observed in infants and young children.
- MCE presents a consistent pattern of brain damage despite diverse underlying causes.
- Understanding the pathogenesis of MCE is crucial for early diagnosis and intervention.
Purpose of the Study:
- To analyze eleven cases of severe multicystic encephalopathy (MCE) in young children.
- To discuss the polyetiologic nature and common pathogenetic mechanisms of MCE.
- To explore the role of anoxia, hypercapnia, and brain edema in MCE pathogenesis.
Main Methods:
- Case series analysis of eleven infants and one child with MCE.
- Review of clinical data, underlying disorders, and pathogenetic mechanisms.
- Literature review to compare findings with previously reported MCE cases.
Main Results:
- Diverse etiologies were identified, including circulatory/respiratory disturbances (5 cases), meningoencephalitis (2 cases), carbon monoxide poisoning (1 case), and meningocerebral angiomatosis (2 cases).
- One case had an obscure etiology following a complicated twin birth.
- A common pathogenetic mechanism, likely involving anoxia, hypercapnia, and brain edema, is proposed for the consistent MCE brain damage pattern.
Conclusions:
- MCE is a polyetiologic condition in early infancy with a characteristic pattern of brain damage.
- The consistent damage pattern suggests a specific reactive mode of the infantile brain to common pathogenetic events.
- Anoxia with hypercapnia and subsequent brain edema are key proposed pathogenetic events in MCE.
Abstract:
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 .