Abstract

Neuropsychologic data suggest an important role for the caudate nucleus (CN) in behavioral impairments in Huntington's disease (HD). These include abnormalities in executive function, egocentric visuospatial representations, communication, and retrieval of declarative memories, changes in personality, and psychiatric disturbances. Animal paradigms of CN lesions support a role for the CN in some of these behaviors. Current theories of basal ganglia function add explanatory value to the role of the CN in these behaviors. A disconnection of the caudate from limbic structures, including the amygdala may account for many nonmotor behaviors observed in HD.

Highlights

  • Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by abnonnal movements, dementia, and psychiatric and personality abnonnalities

  • We have presented a summary of nonmotor behavioral findings in HD as well as a theoretical framework for considering the findings related to dysfunction of the caudate nucleus (CN) or CN-synapsing loops, whereas motor behaviors may be related more closely to dysfunction of the motor loop

  • We have presented animal studies which support an independent role for the CN in behaviors that are very similar to those seen in HD

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Summary

INTRODUCTION

Huntington's disease (HD) is an inherited neurodegenerative disorder characterized by abnonnal movements, dementia, and psychiatric and personality abnonnalities. The neuropathologic changes of HD eventually involve many other structures, including the cortex, the subcortical gray and white matter, and the cerebellar and brainstem nuclei Many of these structures send or receive fibers that synapse in the neostriatum (CN and PUT) (Bruyn et al, 1979; Vonsattel et al, 1985; Roos, 1986; De La Monte et al, 1988). The role of the CN in nonmotor behavior was further refined by descriptions of at least five parallel, anatomically segregated multisynaptic loops which pass from the cortex, through the basal ganglia to the thalamus and return to the frontal lobes (Alexander et al 1986) These tracts are defined by their cortical representations and within the basal ganglia, by target region and neuropeptide.

EXECUTIVE FUNCTION
VISUOSPATIAL FUNCTION
DIGIT SPAN
LANGUAGE AND COMMUNICATION DISORDERS
PERSONALITY AND PSYCHIATRIC FINDINGS
Findings
CONCLUSIONS
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