Experimentelle Sch�digungen von Nervensystem und Muskulatur durch Chlorochin: Modelle verschiedenartiger Speicherdystrophien

Abstract

The neuronal storage dystrophy produced experimentally by chloroquine, contains models for quite a number of well known neurological storage diseases, almost all of which can be traced back to genetically determined defects of various lysosomal enzymes. Within the nervous system the neurones of the posterior root and trigeminal ganglia are predominantly affected in the experimental disease. Electronmicroscopically one finds unusually complex and regionally differing combinations of various types of storage bodies. In rats, the perikarya of posterior root and trigeminal nerve cells contained predominantly the concentrically layered type of membranous cytoplasmic bodies (MCBs) such as seen in infantile amaurotic idiocy. In rabbits, on the other hand, the majority of MCBs consisted of the “zebra body” type, as seen especially in gargoylism. Within the frequent axonal balloonings of these cells there are, in both species, additional small to very large accumulations of glycogen granules or particles usually surrounded by one or several membranes; in type II glycogenosis such glycogen deposits are also stored within neurones. Storage bodies with extensive curvilinear segments are found exclusively within the affected perikarya of neurones in the trigeminal ganglia of rats; they lay either massed together or scattered amongst the layered MCBs. Similar cytosomes are found in some forms of neuronal ceroid-lipofuscinosis.