Jähnel Mathias
Bezirkskrankenhaus Schloss Werneck.
Psychiatr Prax. 2004 Nov;31 Suppl 1:S134-6. doi: 10.1055/s-2004-828455.
This is the rare case of a woman first diagnosed with Huntington's disease at the age of 86. She was first seen at our hospital in 1999 for evaluation of dementia. Prior to her hospitalization, she had been treated with Melperone for restlessness. There was no essential psychopathology other than mild dementia (MMSE 21). A neurological work-up identified generalized involuntary movements and genetic testing revealed 40 CAG repeats. Over the course of the next several years, the patient began to exhibit the full range of symptoms associated with Huntington's disease, such as severe involuntary movements and progressive dementia. In the end, she was bed-ridden and only able to utter a few unintelligible words and she succumbed to an infection in 2003 at the age of 89, almost 4 years after her initial diagnosis of Huntington's disease. This case points to the need to consider Huntington's disease as a differential diagnosis even when evaluating the geropsychiatric population. It is particularly important to isolate involuntary movements which cannot be attributed to tardive dyskinesia in those who have been treated with neuroleptics in the past.
这是一例罕见病例，一名女性在86岁时首次被诊断出患有亨廷顿舞蹈症。1999年她因痴呆评估首次前来我院就诊。住院前，她曾服用美哌隆治疗烦躁不安。除轻度痴呆（简易精神状态检查表评分为21分）外，无其他重要精神病理学表现。神经系统检查发现全身性不自主运动，基因检测显示有40个CAG重复序列。在接下来的几年里，患者开始出现与亨廷顿舞蹈症相关的一系列症状，如严重的不自主运动和进行性痴呆。最终，她卧床不起，只能说出几个难以理解的词，并于2003年，即首次诊断出亨廷顿舞蹈症近4年后，因感染去世。该病例表明，即使在评估老年精神科患者时，也需要将亨廷顿舞蹈症作为鉴别诊断考虑在内。尤其重要的是，在过去接受过抗精神病药物治疗的患者中，要区分出不能归因于迟发性运动障碍的不自主运动。
