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Cortical-basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution,swollen achromasic neurons,and extensive deposition of abnormal tau in neurons and glia.We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms.In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe,amygdala, and hippocampus.In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical- basal ganglionic de3generation,the most severe changes were observed in the left motor cortex and adjacent Broca's area.It is therefore apparent that the histological changes of cortical-basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions.On this basis we conclude that cortical-basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia,particularly when the latter is accompanied by frontal lobe symptomatology,early speech alterations,or parkinsonism. |
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apraxia,speech
cortical-basal ganglionic degeneration
dementia
intellectual deficit
language disorder in adults
memory,impairment of
myoclonus
myoclonus,stimulus sensitive
neuropathology
neuropathology,brain
Parkinson disease
Parkinsonism syndrome
personality change
speech disorder
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