Ideomotor limb apraxia in Huntington's disease: implications for corticostriate involvement.
Ideomotor limb apraxia, a disorder of goal-directed movement, has been attributed to lesions in the frontal and parietal lobes, but the role of subcortical structures is less certain. In order to determine its prevalence in a disorder affecting the basal ganglia and corticostriatal connections, we examined imitation of hand gestures in Huntington's disease (HD) patients. We also assessed the relationship between apraxia and cognitive and motor dysfunction in an effort to better understand the neural underpinnings of apraxia in HD. If damage restricted to the basal ganglia produces ideomotor limb apraxia, then we would expect to find evidence of apraxia in patients who were early in the disease course when selective striatal damage is most common. Such a pattern, however, was not found in our sample. Instead, patients with greater neurological impairment and with a longer duration of disease were more likely than less affected patients to demonstrate apraxia. Apraxia was not related to severity of chorea, but was associated with greater impairment in eye movements, voluntary movements, and verbal fluency. These findings suggest that apraxia in HD results from damage to the corticostriate pathways and the basal ganglia rather than from damage restricted to the basal ganglia.
Hamilton
J M
JM
Department of Neurosciences, University of California, San Diego, CA 92093, USA.
Haaland
K Y
KY
Adair
J C
JC
Brandt
J
J
D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D020240:Apraxia, Ideomotor / Q000209:etiology*; D001479:Basal Ganglia / Q000503:physiopathology*; D016022:Case-Control Studies; D002540:Cerebral Cortex / Q000503:physiopathology*; D005121:Extremities; D005133:Eye Movements / Q000502:physiology; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000191:economics / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009048:Motor Skills / Q000502:physiology; D009483:Neuropsychological Tests; D011597:Psychomotor Performance; D012044:Regression Analysis