[A clinicopathological study of corticobasal degeneration]

To elucidate clinical and pathological features of corticobasal degeneration.

Clinical and image materials of 2 the aged with movement disturbances and dementia were reviewed, their brain tissues were investigated by routine pathological methods, Gallyas-Braak staining and Tau immunohistochemical staining.

Both cases early presented movement disturbance on unilateral extremity including akinesia, rest tremor, postural instability, and then gradually developed rigidity of axial and four extremities, myoclonus, impaired ocular movement, cognitive disturbance from moderate dementia to decorticate state. On SPECT, one case showed hypoperfusion of left frontal-temporal cortices, another presented asymmetric atrophy of frontal-parietal cortices on CT. Main findings on routine pathological examination were prominent atrophy of the frontal-parietal lobe, the basal ganglia, the midbrain and upper pons. The cortical layers of the frontal, parietal and insular lobe showed mild neuronal loss with gliosis and scattered ballooned neurons. Neuron losses and extensive gliosis of basal ganglia and substantia nigra were remarkable. Gallyas-Braak and tau staining showed globous inclusion in remaining neurons of basal ganglia, substantia nigra and locus ceruleus et at, substantive glial cytoskeletol degeneration including astrocyte plaques, tuft-shaped astrocyte were also found in cortex on frontal and parietal lobe and in basal ganglia.

Based on the above-mentioned clinical and pathological features, it may be not difficult to diagnosis corticobasal degeneration.