Greene J D, Patterson K, Xuereb J, Hodges J R
University Neurology Unit, Addenbrooke's Hospital, Cambridge, England.
Arch Neurol. 1996 Oct;53(10):1072-8. doi: 10.1001/archneur.1996.00550100158027.
To describe a patient with pathologically proven Alzheimer disease (AD) who presented with a non-fluent progressive aphasic syndrome.
Longitudinal neuropsychological assessment, structural (magnetic resonance imaging) and functional (single photon emission computed tomography) imaging, and postmortem brain examination.
Memory and cognitive disorders clinic in a tertiary referral hospital.
A 66-year-old man presented with a 5-year history of progressive nonfluent aphasia characterized by marked deficits in phonology and syntax with preservation of everyday abilities. His condition deteriorated rapidly and he died suddenly of a myocardial infarction 12 months later.
Neuropsychological testing revealed mild global intellectual impairment with marked impairment of auditory verbal short-term memory, syntactic, and phonological abilities. His naming errors were predominantly phonological paraphasias. Magnetic resonance imaging scans showed left perisylvian atrophy and results of a Tc 99m hexamethyl-propyleneamine-oxime single photon emission computed tomographic scan were normal. Postmortem pathological examination revealed typical AD pathological features with atypical distribution, involving predominantly perisylvian language areas, but sparing the medial temporal lobe.
The language deficits in AD, which have received considerable attention, are thought to involve predominantly lexicosemantic processes. When AD presents as a relatively isolated language disturbance, the aphasia is usually of the fluent anomic type. To our knowledge, our patient represents the first fully documented case of progressive nonfluent aphasia with pathologically verified AD.
描述一名经病理证实为阿尔茨海默病（AD）且表现为非流畅性进行性失语综合征的患者。
纵向神经心理学评估、结构（磁共振成像）和功能（单光子发射计算机断层扫描）成像以及死后脑检查。
一家三级转诊医院的记忆与认知障碍诊所。
一名66岁男性，有5年进行性非流畅性失语病史，其特征为语音和句法明显缺陷，但日常能力保留。他的病情迅速恶化，12个月后因心肌梗死突然死亡。
神经心理学测试显示轻度整体智力损害，伴有听觉言语短期记忆、句法和语音能力明显受损。他的命名错误主要是语音性错语。磁共振成像扫描显示左侧颞周萎缩，锝99m六甲基丙烯胺肟单光子发射计算机断层扫描结果正常。死后病理检查显示典型的AD病理特征，但分布不典型，主要累及颞周语言区，而内侧颞叶未受累。
AD中的语言缺陷受到了相当多的关注，一般认为主要涉及词汇语义过程。当AD表现为相对孤立的语言障碍时，失语通常为流畅性命名性失语类型。据我们所知，我们的患者是首例有病理证实的AD伴进行性非流畅性失语的完整记录病例。
