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Washington University Experience | NEURODEGENERATION | Huntington Disease | 17A0 Case 17 History
Case 17 History ---- This man with a strong family history of HD was first evaluated in the MDC at age 47. At age 44 he developed increasing irritability; intermittent agitation and occasional lashing out at family members including his wife. This was very different from his premorbid personality. At age 45, he started to mumble when speaking. About that time, he would awaken at night with his mind racing. His wife noticed mild chorea but he was unaware of such movements. Exam revealed hypomanic behavior with pressure speech and generalized chorea at age 47. Over the next 10 years, he had progressive irritability, violent behavior, persisting denial of difficulties and progressive chorea with poor postural stability. He also had increasing cognitive impairment with substantial impairment of activities of daily living by age 51. He became increasingly impulsive. In the last few years of his life, he had more trouble with hand coordination and could no longer tie a fishing fly. Chorea became substantial and apraxia also became prominent. Balance was impaired due to truncal instability. He began to fall and in the last year, struck his head during a fall (while fishing) and sustained a subdural hematoma. He then had increasing difficulty with ambulation, poor swallowing and increased dementia. He was on hospice care at the end of his life and died from inanition and dehydration in June 2013. Medications included quetiapine, Celebrex, Depakote, and tamsulosin.
