(1) Division of Neuropathology, Department of Pathology, Montefiore Hospital and Medical Center, 111 East 210th Street, 10467 Bronx, New York, USA;(2) Albert Einstein College of Medicine, 10467 Bronx, New York
Abstract:
Summary The neurofibrillary tangles found in the neurons of a case of Steele-Richardson-Olszewski syndrome were studied by electron microscopy. Both the flame-shaped and globose type of tangles were present. The neurofibrillary tangles were composed of bundles of straight tubules measuring approximately 150 Å in diameter. This ultrastructural appearance of the tangles in Steele-Richardson-Olszewski syndrome is different from the appearance of tangles found in other neurological disorders and is probably indicative of intraneuronal accumulation of a new type of fibrous protein.This investigation received financial support from the World Health Organization.