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Washington University Experience | NEURODEGENERATION | Huntington Disease | 9A0 Case 9 History
Case 9 History The patient was a 57yo female with a history of Huntington's disease. The patient has a strong family history of Huntington's disease including her mother and four of her mother's nine siblings. They all developed Huntington's in their 50s with jerky movements and progressive cognitive decline. At the age of 48 (1991) this patient began to develop depressive symptoms with low mood, difficulty sleeping and feelings of helplessness. She was treated with Nortriptyline and Buspirone at this time. She began to experience episodes of chorea approximately in 1995. She also began experiencing intellectual impairment in 1995. She was forced to quit work in 1996 due to cognitive disability. She was being treated with Depakote for her chorea. She was also started on Haloperidol in August of 1999 to attempt to control her irritability and aggressiveness. She moved to an assisted living facility in the summer of 1999. She had progressive problems with her ambulation as well as difficulty with feeding herself and activities of daily living. On her last office examination in July of 2000, she was noted to be oriented to person only and had difficulty naming objects as well as orientation questions. The patient continued her physical and mental decline and was recently admitted to BJH with aspiration pneumonia and was treated with IV antibiotics and transferred back to the nursing home. She also had a G-tube placed at her last hospitalization for aspiration. She was found unresponsive in January 2001 at the nursing home, requiring intubation and underwent cardiopulmonary resuscitation as per ACLS protocol. Her vital signs were stabilized and she was transferred to BJWCH. She was placed in the ICU there for monitoring. The reason for her arrest was unknown, but it was thought probably secondary to aspiration or pneumonia. The situation was discussed with the family who decided to discontinue life support. She was pronounced dead in January 2001. ---- At autopsy the weight of the unfixed brain is 1300g.