UNUSUAL CASE OF CORTICOBASAL DEGENERATION WITH TAU GALLYAS-POSITIVE NEURONAL AND GLIAL TANGLES/

A 74-year-old woman with corticobasal degeneration (CBD) had a 9-year history of progressive loss of strength and rigidity of her right hand and then arm, followed by speech difficulties, dyskinesia, rigidity, spasticity and weakness of the ipsilateral lower limb, ultimately also involving the apposite side. She later developed supranuclear gaze pa lsy. Her memory remained intact during most of the duration of her dis ease. Laboratory tests and anti-Parkinsonian medications were not help ful. At autopsy, frontal lobe atrophy, discoloration of putamen (Pt) a nd pallor of substantia nigra (Sn) were observed. Neuronal loss and gl iosis were extensive in motor cortex and milder in frontal cortex, abr uptly ending at the central sulcus and junction of cingulate gyrus. '' Achromatic'' neurons were present. Neuronal loss and gliosis were seen in Pt and Sn and corticobasal inclusions in Sn. Numerous Gallyas/tau- positive, Bielschowsky/ubiquitin-negative coil, sickle, or coma-shaped tangles and thread-like processes were found in affected cortex, Pt a nd Sn. Some of the tangles were in neurons, but most occurred in astro glia, and their processes. The presence of Gallyas/tau-positive glia i n CBD may have the same diagnostic significance as in progressive supr anuclear palsy, analogous to the argyrophilic ubiquinated inclusions i n oligodendroglia in multisystem atrophy. We suggest that in CBD: (1) cytoskeletal protein metabolism in neurons and glia can simultaneously be perturbed in certain neurodegenerative diseases, and (2) the astro cytosis in CBD may not be simply a reactive process but an integral pa rt of the disease.