Abstract
Huntington’s disease (HD) is a fatal inherited neurological disorder, the genetic basis of which has recently been identified as an expanded trinucleotide repeat in a previously unknown gene (the normal function of which remains mysterious) on chromosome 4 (1). The disease is characterized pathologically by a primary progressive loss of medium spiny projection neurons within the neostriatum (caudate nucleus and putamen) in addition to fibrillary astrocytosis. In later stages of the disease the pathology spreads beyond the striatum itself to affect other cortical and subcortical systems, although predominantly those that are afferent and efferent to the striatum. HD is traditionally viewed as a movement disorder, and is typically characterized by dementia and psychiatric symptoms in addition to the more conspicuous choreic movements. This composition of impairments suggests a striatal influence in many responserelated functions, mirroring the diversity of its neocortical afferents.
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Brasted, P.J., Döbrössy, M.D., Eagle, D.M., Nathwani, F., Robbins, T.W., Dunnett, S.B. (2000). Operant Analysis of Striatal Dysfunction. In: Emerich, D.F., Dean, R.L., Sanberg, P.R. (eds) Central Nervous System Diseases. Contemporary Neuroscience. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-691-1_14
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