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J Neurophysiol 91: 230-238, 2004. First published September 17, 2003; doi:10.1152/jn.00557.2003
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Hereditary Cerebellar Ataxia Progressively Impairs Force Adaptation During Goal-Directed Arm Movements

Matthias Maschke1,4, Christopher M. Gomez2, Timothy J. Ebner3 and Jürgen Konczak1,2

1 Sensorimotor Control Laboratory, University of Minnesota, Minneapolis, Minnesota 55455 2 Department of Neurology, University of Minnesota, Minneapolis, Minnesota 55455 3 Department of Neuroscience, University of Minnesota, Minneapolis, Minnesota 55455 4 Department of Neurology, University of Duisburg-Essen, 45122 Essen, Germany

Submitted 10 June 2003; accepted in final form 10 September 2003

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 aftereffects 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.

Address for reprint requests and other correspondence: M. Maschke, Dept. of Neurology, University of Duisburg-Essen, Hufelandstr. 55, 45122 Essen, Germany (E-mail: matthias.maschke{at}uni-essen.de).




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