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Huntington disease (HD) is a progressive, inherited neurodegenerative disorder characterized by motor, cognitive, and behavioral features, with symptom onset most often in mid-adulthood. HD is caused by a trinucleotide repeat expansion in the huntingtin gene (HTT), encoding the protein huntingtin, resulting in a mutant protein containing an expanded polyglutamine sequence. Although more widespread neuronal loss is evident in advanced disease, neuropathologic changes in early HD are most prominent in the striatum with loss of striatal medium spiny neurons (MSN).1 There is good evidence that striatal changes precede the onset of clinical symptoms with MRI indications of striatal atrophy occurring 10 or more years prior to clinical onset.2 HD is inexorably progressive, leading to death approximately 20 years after symptom onset. Attempts to alter this natural history with disease-modifying therapies (DMTs) have been unrewarding but there are some exciting DMT possibilities on the horizon.
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