Corticocerebellothalamic degeneration with chromatolytic neuronal swelling: a light and electron microscopic case...

A 50-year-old woman showing psychoorganic syndrome, cerebellar symptoms, choreatic involuntary movements, Romberg's sign and epileptic fits over a period of 14 years, was neuropathologically studied. The calcarine and postcentral cortex, the medio-dorsal nucleus of the thalamus and the cerebellar cortex were affected with neuronal loss and astrocytic gliosis. Neurons in various regions showed chromatolytic neuronal swelling. Ultrastructurally, these chromatolytic neurons contained a large hollow core with numerous mitochondria, dispersed rough endoplasmic reticulum, degenerated cisternal structures and a few neurofilaments. Some neuronal mitochondria showed membranous trans-somal bridges composed of about 60 A parallel filaments with a spacing of about 80 A.