Naasan George, Shany-Ur Tal, Sidhu Manu, Barton Cynthia, Ketelle Robin, Shdo Suzanne M, Kramer Joel H, Miller Bruce L, Seeley William W
a Memory and Aging Center, Department of Neurology , University of California San Francisco , San Francisco , CA , USA.
Neurocase. 2019 Feb-Apr;25(1-2):26-33. doi: 10.1080/13554794.2019.1604973. Epub 2019 Apr 22.
Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.
皮质基底节综合征和路易体痴呆是具有独特和重叠特征但病理基础不同的临床表现。我们报告了一例80岁男性患者，其表现为失用症、强直、运动迟缓、右臂肌阵挛、有10年可能的快速眼动睡眠行为障碍病史，后来出现视幻觉。尸检时，他具有皮质基底节变性的病理特征，且路易体病仅限于脑干。本报告强调了在临床表现难以分类时考虑并存病理的重要性，并表明路易体痴呆的显著特征可能源于仅限于脑干的病理改变。
