Corticobasal degeneration: Widespread argentophilic threads and gliain addition to neurofibrillary tangles. Similarities of cytoskeletalabnormalities in corticobasal degeneration and progressivesupranuclear palsy


T. Takahashi
Bok Engelsk 1996
Utgitt
1996
Omfang
Side 66- 77
Opplysninger
A 57-year-old man had exhibited cortical sensory disturbance,rigidity, spasticity, dementia, alien hand, grasp reflex,supranuclear ophthalmoplegia, pseudobulbar palsy, and neck dystoniafor 4 years. Histological examination of autopsied specimens revealedneuronal loss in the cerebral cortex, with ballooned neurons,subthalamic nucleus, substantia nigra, basal ganglia, midbraintegmentum, and the thalamus. There were neurofibrillary tangles inthe subthalamic nucleus and the substantia nigra. Gallyas-Braaksilver impregnation demonstrated numerous argentophilic tangles,threads, and a few argentophilic glia in the cerebral cortex,subcortical white matter, particularly in the precentral gyrus,subcortical nuclei, and the brainstem. These argentophilic structureswere largely positive for tau, and negative for ubiquitin, pairedhelical filaments, and phosphorylated neurofilament.Ultrastructurally, 15-nm-wide straight tubules were observed in theneurons of the substantia nigra, globus pallidus, and the precentralcortex, coexisting with a few twisted tubules periodicallyconstricted at 160- to 230-nm intervals. It was conclusively shownthat Gallyas- and tau-positive cytoskeletal abnormalities occurredwidely in brain of corticobasal degeneration. Both distribution andmorphology of abnormal phosphorylated tau protein in corticobasaldegeneration appear to resemble these features in progressivesupranuclear palsy. These findings suggest a common cytoskeletaletiopathological significance in corticobasal degeneration andprogressive supranuclear palsy.
Emner

Bibliotek som har denne