Gerstner Elizabeth, Lazar Ronald M, Keller Christian, Honig Lawrence S, Lazar Gloria S, Marshall Randolph S
Columbia University College of Physicians and Surgeons, New York, NY, USA.
Cogn Behav Neurol. 2007 Mar;20(1):15-20. doi: 10.1097/WNN.0b013e31802b6c45.
To present the case of a man with progressive speech loss and other clinical features and diagnostic tests consistent with fronto-temporal dementia but whose postmortem neuropathologic findings revealed Alzheimer disease (AD).
Progressive apraxia of speech presents without true language abnormalities, usually seen with frontal lesions and not associated with AD pathology.
We describe the clinico-pathologic case of an 87-year-old man with progressive loss of speech function and present the prospective presentation of his syndrome using structural (magnetic resonance imaging) and metabolic (positron emission tomography) neuro-imaging studies, neuropsychologic testing, and pathology.
His syndrome was characterized over the first 6 to 9 years by progressive deterioration of speech production, alteration of mood, and dysphagia but near normal language, memory, and visual-spatial function. At 8 years, fluorodeoxyglucose-positron emission tomography showed largely frontal metabolic abnormality. Over his final 1(1/2) years, he was mute and withdrawn. Neuropathologic findings showed neuritic plaques and neurofibrillary tangles, but no signs of frontotemporal dementias such as Pick bodies or ubiquitinated tau-negative inclusions.
There can be overlap in the presentation of fronto-temporal dementia and AD despite the disparate pathologic bases of the underlying diseases. It has yet to be determined how to differentiate these diseases in such variant presentations and whether such atypical AD syndromes are equally amenable to standard therapies for AD.
报告一例患有进行性言语丧失及其他临床特征和诊断检查结果符合额颞叶痴呆，但死后神经病理学检查发现为阿尔茨海默病（AD）的男性病例。
进行性言语失用症在无真正语言异常的情况下出现，通常见于额叶病变，且与AD病理无关。
我们描述了一名87岁男性进行性言语功能丧失的临床病理病例，并通过结构（磁共振成像）和代谢（正电子发射断层扫描）神经影像学研究、神经心理学测试及病理学检查展示了其综合征的前瞻性表现。
在最初的6至9年里，他的综合征表现为言语表达逐渐恶化、情绪改变和吞咽困难，但语言、记忆和视觉空间功能基本正常。8年时，氟脱氧葡萄糖-正电子发射断层扫描显示主要为额叶代谢异常。在最后的1.5年里，他变得沉默寡言、离群索居。神经病理学检查结果显示有神经炎性斑块和神经原纤维缠结，但没有额颞叶痴呆的迹象，如Pick小体或泛素化tau阴性包涵体。
尽管潜在疾病的病理基础不同，但额颞叶痴呆和AD的临床表现可能存在重叠。目前尚不清楚如何在这些变异表现中区分这些疾病，以及这种非典型AD综合征是否同样适用于AD的标准治疗方法。
