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Washington University Experience | NEURODEGENERATION | Huntington Disease | 6A0 Case 6 History
Case 6 History ---- This 42yo female was first recognized as having Huntington's disease approximately 13 years prior to death. She presented with primarily affective symptoms initially treated with electroconvulsive therapy and antidepressant medications. She subsequently developed choreiform movements which were unsuccessfully treated with Haldol and Prolixin. In January of 1980 she developed increasing difficulty with swallowing and choking on liquids. At that time she was able to walk with assistance but was unable to eat or dress independently. She showed progressive deterioration during her last year of life with wild flailing movements of the extremities, inability to walk, altered affect and worsening dysphagia. She was readmitted to BJC in September and October with choking and dyspnea attributed to pneumonitis for which she was treated with Ampicillin. In addition, she was treated with Tetrabenazine for her movement disorder and Amitriptyline and Meprobamate for affective symptoms. Her family history is positive for Huntington's disease in her father who died at age 54 and possibly in a maternal grandfather who committed suicide at age 55. She has four children, none of which are known to be affected. She was oriented to person and place but not to time. Affect was labile and frequently agitated. Her memory was intact. Speech consisted of grunting verbalizations which were difficult to understand. She was, however, able to indicate "yes" or "no" by shaking her head. 'l'he patient was treated with Ampicillin and discharged to home where she was found dead shortly after. ---- At autopsy her unfixed brain weighed 970g.
