1981 Volume 12 Issue 2 Pages 123-129
Pathological findings of a patient with progressive supranuclear palsy (PSP) were reported. The patient was a 77-year-old male, who showed, during about 12 years of his illness, typical symptoms of PSP, such as dysarthria, supranuclear opthalmoplegia to all directions, axial dystonic rigidity with extention of the neck and hyperreflexia. In addition, he showed difficulty in eyelid opening, which was diagnosed as so-called "apraxia of lid opening" by EMG using surface electrodes. CT revealed obvious dilatation of the quadrigeminal and ambient cisterns indicating atrophy of the brain stem. On autopsy, the brain weighed 1220g, and seemed almost normal except for slight atrophy of the brain stem. Microscopic examination demonstrated marked neuronal loss and gliosis especially in the globus pallidus, nucleus subthalamicus and substantia nigra. A lot of neurofibrillary tangles were observed in the superior colliculus, periaqueductal gray matter, substantia nigra and locus caeruleus. Eosinophilic granules were found in the globus pallidus and substantia nigra. In electron microscopic studies, it should be stressed that the neurofibrillary tangles in this case were composed of bundles of 100Å neurofilaments. This finding is different from previous reports in which 130-150Å straight or twisted neurotubules were described as the components.
