A NEUROPATHOLOGIC STUDY OF LONG-TERM, ECONOMO-TYPE POSTENCEPHALITIC PARKINSONISM WITH A PROLONGED CLINICAL COURSE

In this report, the neuropathologic features of five autopsied cases o f postencephalitic parkinsonism of the Economo-type (PEPE) with a mean age of 66.6 years and a mean duration of the illness of 53.6 years ar e described. All five patients had presented with personality changes and severe parkinsonism. In addition, four patients had also had ocula r symptoms. A pronounced chronic progression of the symptoms character ized all five cases. Active degenerating lesions were found in the sub stantia nigra (patients 3, 4 and 5) and the oculomotor nucleus (patien t 5) which might explain the clinical observation of chronic active di sease in these patients. We found that the intraneuronal neurofibrilla ry tangles (NFT) were immunoreactive to paired helical filaments (PHF) , tan and ubiquitin; but ghost tangles demonstrated immunoreactivity o nly to glial fibrillary acid protein (GFAP). The ghost tangles consist ed of dispersed bundles of abnormal tubules, and electron-dense glial filaments would surround and occasionally invade the ghost tangles. Th e present study suggests that NFT in PEPE are similar in their immunoh istochemistry and ultrastructure to those observed in the case of Alzh eimer-type dementia.