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Corticobasal ganglionic degeneration

A 78 year-old woman presented with two years of unsteady gait and difficulty using her hands. Her exam was notable for prominent apraxia of both upper extremities and cortical sensory loss (i.e., agraphesthia, astereognosia). She had increased tone in both legs and a slow shuffling gait.

Show the Parietal Atrophy               Show the Enlarged Superior Frontal Sulcus

(Left) Axial; (Right) Sagittal T1-weighted MRI scans. Note the prominent atrophy in the parietal areas bilaterally with widening of the adjacent sulci. In addition, note the slight asymmetry of the superior frontal sulcus on the left compared to the contralateral side. This patient had corticobasal ganglionic degeneration, a rare degenerative condition where the cortex ( usually the parietal lobe most prominently) is affected along with the basal ganglia. Patients often present with prominent apraxia and in marked cases, will display the "alien hand" syndrome, where their hand feels disconnected from their body.

Revised 04/18/06.
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