*Cerebral Function Unit §Department of Neuroradiology, Greater Manchester Neurosciences Centre, Salford †Mental Health and Neurodegeneration Research Group, School of Community-Based Medicine ‡School of Cancer and Enabling Sciences, University of Manchester, Manchester ∥Department of Neurology, Royal Preston Hospital, Preston ¶National CJD Research and Surveillance Unit, Western General Hospital, Edinburgh, UK.
Alzheimer Dis Assoc Disord. 2013 Oct-Dec;27(4):384-6. doi: 10.1097/WAD.0b013e318260ab27.
Progressive non-fluent aphasia (PNFA) is typically associated with pathological changes consistent with frontotemporal lobar degeneration. A 65-year-old male presented with effortful speech, markedly impaired naming and features of speech apraxia, consistent with PNFA. Perceptuospatial function, calculation and executive function were intact. Brain SPECT showed left perisylvian hypoperfusion. He deteriorated profoundly over the subsequent eight months, with appearances on diffusion-weighted magnetic resonance imaging typical of sporadic Creutzfeldt-Jakob disease, which was confirmed pathologically at postmortem examination. While the presence of PNFA with speech apraxia is thought to predict underlying tauopathy, sporadic Creutzfeldt-Jakob disease may mimic this presentation and present in a highly circumscribed form not previously described.
进行性非流利性失语症（PNFA）通常与符合额颞叶变性的病理变化相关。一位 65 岁男性表现为费力性言语、明显的命名障碍和言语失用特征，符合 PNFA。知觉空间功能、计算和执行功能完好。脑 SPECT 显示左侧大脑皮质下灌注不足。在随后的八个月中，他的病情急剧恶化，弥散加权磁共振成像表现为散发性克雅氏病的典型表现，在尸检中得到病理证实。虽然伴有言语失用的 PNFA 被认为预示着潜在的 tau 病，但散发性克雅氏病可能会模仿这种表现，并以以前未描述的高度局限性形式出现。
