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Contrary to popular belief, receiving a diagnosis of a devastating fatal disease does not exacerbate, and may even alleviate, the risk of suicide. Suicidal ideation was examined in 4,171 individuals in the Huntington Study Group database. Participants were grouped according to a standardized neurological examination from 0 (i.e., normal examination) to 3 (definite Huntington’s disease). Patients with an unequivocal diagnosis of Huntington’s disease were further divided by stage of disease, from stage 1 (early) to stage 5 (end stage). Findings showed that the frequency of suicidal ideation doubled from 9.1% in at-risk persons with a normal neurological examination to 19.8% in at-risk persons with soft neurological signs and increased to 23.5% in persons with “possible Huntington’s disease.” In persons with a diagnosis of Huntington’s disease, 16.7% had suicidal ideation in stage 1, and 21.6% had suicidal ideation in stage 2, whereas the proportion of Huntington’s disease patients with suicidal ideation diminished thereafter. Findings suggest two critical periods for increased risk of suicide in Huntington’s disease. The first critical period is immediately before receiving a formal diagnosis of Huntington’s disease, and the second is in stage 2 of the disease, when independence diminishes. Although the underlying mechanisms of suicidal ideation in Huntington’s disease are poorly understood, it is critical for health care providers to be aware of periods during which patients may be at an increased risk.