Corticobasal degeneration(CBD) is a
neurodegenerative disorder characterised clinically by
apraxia, cortical sensory loss, alien limb,
dementia, oculomotor abnormalities,
dysarthria, postural instability, akinesia, rigidity, and pyramidal signs. Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. We reported a case of
corticobasal degeneration of which praxic impairments were improved by administration of
amantadine. The patient was a 63-year-old right-handed woman. She showed marked dysfunction including rigidity,
limb kinetic apraxia, cortical sensory loss,
ideomotor apraxia, and
dressing apraxia. A brain MRI scan revealed bilateral cortical
atrophy centered in the postcentral gyrus, more pronounced in the left hemisphere than the right. A SPECT scan showed a decrease in blood flow in the temporo-parieto-occipital regions, more pronounced in the left hemisphere than the right. An EEG showed a diffuse slowness.
L-dopa had no effect on the symptoms of rigidity,
limb kinetic apraxia, cortical sensory loss,
ideomotor apraxia, and
dressing apraxia. By administration of
amantadine, rigidity and cortical sensory loss did not improve, but some praxic impairments, such as
dressing apraxia and
ideomotor apraxia, and the EEG improved. Upon withdrawal of
amantadine, the improved symptoms deteriorated.
Amitriptyline did not improve the deteriorated symptoms. After
amantadine was re-administered, the same praxic impairments and the EEG improved again. This suggested that administration of
amantadine had some effect on certain praxic impairments and the EEG.