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Differential Diagnosis

Diagnostic evaluation may include a series of tests to eliminate other disorders with similar symptoms. For example, certain autosomal dominant neurodegenerative disorders closely mimic HD. These include neuroacanthocytosis and dentatorubropallidoluysian atrophy.

HD must also be differentiated from other disorders or conditions associated with chorea, such as Wilson disease; drug-induced tardive dyskinesia; Sydenham's chorea; systemic lupus erythematosus; or senile chorea, a symptom complex primarily characterized by the development of chorea after age 60. Although some patients with senile chorea may have neurodegenerative changes of the caudate nuclei, there is typically no family history of HD. Some investigators indicate that the disorder may result from a different genetic mutation than that seen in HD; however, others suspect that it may be a late-onset HD variant.