CLOSE RELATIONSHIP BETWEEN SPONGIFORM CHANGE AND UBIQUITIN-POSITIVE GRANULAR STRUCTURES IN DIFFUSE LEWY BODY DISEASE

The origin of spongiform change seen in diffuse Lewy body disease (DLB D) cases was elucidated, as compared with Alzheimer-type dementia (ATD ) cases, Spongiform change was composed of numerous vacuoles in the ne uropil, predominantly in layers II-IIIab of the transentorhinal cortex and in the intermediate area of the accessory basal amygdaloid nucleu s. The distribution of spongiform change was identical to that of ubiq uitin-positive granular structures (UPG) in non-demented cases. The de gree of spongiform change was correlated with loss of the large pyrami dal neurons in layers IIIc and V of the transentorhinal cortex and wit h the disappearance of their ubiquitin-positive granular processes. Wi th electron microscopy, the early vacuoles of spongiform change appear ed to arise in the presynaptic terminals as well as in the postsynapti c terminals and dendritic processes. These findings indicate that the vacuoles derive from degeneration of terminal axons of the large pyram idal neurons. The occurrence of spongiform change characteristic to DL BD suggests that the large pyramidal neurons degenerate more rapidly i n DLBD than in ATD. (C) 1997 Elsevier Science B.V.