[Senile subcortical neurofibrillary degeneration with the presence of twisted tubules and straight filaments. Atypical form of progressive supranuclear paralysis].
A case with wide-spread subcortical neurofibrillary changes, cell loss and gliosis in a 81 year old man is described. The most affected areas were both pallida, Luys subthalamic bodies, the substantia nigra and the denate nuclei of the cerebellum. The cerebral cortex contained only a few senile plaques. We interpreted this case as a variety of progressive supranuclear palsy despite the lack of the typical supranuclear ophtalmoplegia and of superior colliculi involvement. In the subthalamic body and in the substantia nigra fibrillary tangles were investigated ultrastructurally and found to consist of two kinds of fibrillary material: straight fine filaments of 100 A and twisted tubules with an average diameter of 200 A. We did not see 150 A straight filaments like those found in some cases of progressive supranuclear palsy. The possible significancy of each kind of neuro-fibrillary tangles and of their simultaneous occurrence in the subcortex of this case is discussed.
D000368:Aged; D001921:Brain / Q000648:ultrastructure*; D010244:Bulbar Palsy, Progressive / Q000473:pathology*; D002529:Cerebellar Nuclei / Q000648:ultrastructure; D005917:Globus Pallidus / Q000648:ultrastructure; D006801:Humans; D008297:Male; D008870:Microtubules / Q000648:ultrastructure*; D009454:Neurofibrils / Q000648:ultrastructure*; D013378:Substantia Nigra / Q000648:ultrastructure; D013787:Thalamic Nuclei / Q000648:ultrastructure
Dégénérescence neurofibrillaire sous-corticale sénile avec présence de tubules contournés et de filaments droits. Forme atypique de la paralysie supranucléaire progressive.