Idiopathic nonarteriosclerotic cerebral calcification (Fahr's disease): an electron microscopic study

A case of idiopathic nonarteriosclerotic cerebral calcification was studied post mortem by histochemical and scanning and transmission electron microscopic methods. Calcification was found bilaterally in the basal ganglia, cerebral cortex, granular layer and white matter of the cerebellum. Histochemical examination revealed that deposits were composed of a mixture of glycoproteins, mucopolysaccharides, calcium salts and iron. Transmission electron microscopy revealed minute deposits mainly in the cytoplasm of adventitial cells of blood vessels and sometimes in the cytoplasmic processes of glial cells. Scanning electron microscopy showed that some of the spherical and hemispherical bodies, which were formed in the adventitial cells of blood vessels, were connected with filamentous cytoplasmic processes of surrounding cells. Small uncalcified deposits occurring in the cytoplasm of the adventitial cells had subsequently had minerals deposited in them. Some kind of impairment of the pericytes may play an important role at the onset of this disease.