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* To whom correspondence should be addressed. E-mail: matthias.maschke{at}uni-essen.de.
We investigated how humans with hereditary cerebellar degeneration (spinocerebellar ataxia (SCA) type 6 and 8, n=9) and age- and sex-matched healthy controls (n=9) adapted goal-directed arm movements to an unknown external force-field. We tested whether learning could be generalized to untrained regions in the workspace, an aspect central to the idea of an internal model, and if any learning could be retained. After removal of the force-field, SCA patients showed little or no learning-related after-effects indicating that repeated force-field exposure never led to successful force compensation. In contrast, healthy control subjects quickly adapted their movements to the new force-field. The difference in force-adaptation was significant for movements to targets that required both the shoulder and elbow joint (p<0.001). Moreover, the generalization of learned movements to targets outside the learned workspace was prevented by the cerebellar degeneration (p<0.01). Retention of force-adaptation was significantly lower in SCA patients (p=0.003). The severity of ataxia in SCA patients correlated negatively with the extent of learning (r=-0.84, p=0.004). Our findings imply that progressive loss of cerebellar function gradually impairs force-adaptation. The failure to generalize learning suggests that cerebellar degeneration prevents the formation of an internal representation of the limb dynamics.
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