Alzheimer Abnormalities of the Amygdala With Kluver-Bucy Syndrome Symptoms
Alzheimer Abnormalities of the Amygdala With Kluver-Bucy Syndrome Symptoms
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DOI：
10.1001/archneurol.2008.517
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发表时间：
2009-01-01
影响因子：
--
通讯作者：
DeCarli, Charles
中科院分区：
文献类型：
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作者：
Kile, Shawn J.;Ellis, William G.;DeCarli, Charles
背景：在阿尔茨海默病患者的杏仁核中观察到神经元缠结和β-淀粉样斑块。杏仁核异常的不成比例的丰富可能会导致类似于Kluver-Bucy综合征的行为症状。目的：描述阿尔茨海默病的非典型行为表现，并回顾有关该主题的文献。设计：病例研究。设置：门诊专科诊所。患者：一名70岁男性，具有进行性行为症状，包括口功能亢进、性欲亢进、过度变态、视觉失认、食欲过盛，主要结果：临床病理学、脑影像学和神经病理学。结果：病理诊断为阿尔茨海默病，外侧杏仁核有大量缠结和斑块。结论：该病例代表了阿尔茨海默病的一种变体，具有明显的杏仁核异常和Kluver-误诊为额颞叶痴呆的Bucy表型。临床和影像学检查结果，可能有助于准确的诊断进行审查。
Background: Neurofibrillary tangles and beta-amyloid plaques have been observed in the amygdala in Alzheimer disease. A disproportionate abundance of this abnormality in the amygdala may cause behavioral symptoms similar to Kluver-Bucy syndrome.Objectives: To describe an atypical behavioral presentation of Alzheimer disease and to review the literature on the subject.Design: Case study.Setting: Outpatient specialty clinic.Patient: A 70-year-old man with progressive behavioral symptoms of hyperorality, hypersexuality, hypermetamorphosis, visual agnosia, hyperphagia, and apathy who died at age 77 of asphyxiation on a foreign object.Main Outcome Measures: Clinical symptomatology, brain imaging, and neuropathology.Results: The pathologic diagnosis was Alzheimer disease with abundant tangles and plaques in the lateral amygdala.Conclusions: This case represents a variant of Alzheimer disease with prominent amygdala abnormalities and a Kluver-Bucy phenotype that was misdiagnosed as frontotemporal dementia. Clinical and imaging findings that may aid in accurate diagnosis are reviewed.