Corpora-amylacea and the family of polyglucosan diseases

Abstract

The history, characters, composition and topography of corpora amylacea (CA) in man and the analogous polyglucosan bodies (PGB) in other species are documented, noting particularly the wide variation in the numbers found with age and in neurological disease. Their origins from both neurons and glia and their probable migrations and ultimate fate are discussed. Their presence is also noted in other organs, particularly in the heart. The occurrence in isolated cases of occasional `massive' usually focal accumulations of similar polyglucosan bodies in association with certain chronic neurological diseases is noted and the specific conditions Adult Polyglucosan body disease and type IV glycogenosis where they are found throughout the nervous system in great excess is discussed. The distinctive differences of CA from the PGB of Lafora body disease and Bielschowsky body disease are emphasised. When considering their functional roles, a parallel is briefly drawn on the one hand between normal CA and the bodies in the polyglucosan disorders and on the other with the lysosomal system and its associated storage diseases. It is suggested that these two systems are complementary ways by which large, metabolically active cells such as neurons, astrocytes, cardiac myocytes and probably many other cell types, dispose of the products of stressful metabolic events throughout life and the continuing underlying process of aging and degradation of long lived cellular proteins. Each debris disposal system must be regulated in its own way and must inevitably, a priori, be heir to metabolic defects that give rise in each to its own set of metabolic disorders.