Caine E D, Hunt R D, Weingartner H, Ebert M H
Arch Gen Psychiatry. 1978 Mar;35(3):377-84. doi: 10.1001/archpsyc.1978.01770270127013.
The neuropsychiatric syndrome of Huntington's disease is outlined in this report with an emphasis on the cognitive deficits that lend themselves to future neurobehavioral research. Eighteen patients without disabling cognitive or psychiatric symptoms were evaluated for a period of 3 to 15 weeks, with assessment of their cognitive disorder, psychiatric, and neurological symptoms. Neuropsychological examination included repeated mental status examination, the Wechsler Adult Intelligence Scale (WAIS), and, for some, parietal lobe testing. In addition to suffering from a loss of finely detailed memories, patients demonstrated impaired organizing, sequencing, planning, and recalling of information on request. On the WAIS, mean verbal and performance scores were not significantly different. Neuropsychological findings suggested that the Huntington's disease pattern of cognitive impairment is not initially diffuse and homogeneous, but characterized by a relative sparing of several higher cortical functions. Many patients had increased irritability and labile affect. The similarity of Huntington's disease to frontal lobe syndromes is also discussed.
本报告概述了亨廷顿舞蹈症的神经精神综合征，重点关注有助于未来神经行为研究的认知缺陷。对18名没有致残性认知或精神症状的患者进行了为期3至15周的评估，评估了他们的认知障碍、精神和神经症状。神经心理学检查包括重复的精神状态检查、韦氏成人智力量表（WAIS），部分患者还进行了顶叶测试。除了精细细节记忆丧失外，患者在信息的组织、排序、规划和按需回忆方面也表现受损。在WAIS测试中，平均言语和操作得分没有显著差异。神经心理学研究结果表明，亨廷顿舞蹈症的认知障碍模式最初并非弥漫性和均匀性的，而是以几种高级皮质功能相对保留为特征。许多患者易怒且情绪不稳定。还讨论了亨廷顿舞蹈症与额叶综合征的相似性。
