Rippon G A, Boeve B F, Parisi J E, Dickson D W, Ivnik R I, Jack C R, Hutton M, Baker M, Josephs K A, Knopman D S, Petersen R C
Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Neurocase. 2005 Jun;11(3):204-11. doi: 10.1080/13554790590944753.
Progressive supranuclear palsy (PSP) is typically manifested by vertical supranuclear gaze palsy, frequent falls early in the disease course, axial rigidity and poor response to levodopa. Prominent anterograde memory dysfunction with subsequent impairment in other cognitive domains is characteristic of Alzheimer's disease (AD). No clear clinical syndrome has been identified in argyrophilic grain disease (AGD). Frontotemporal dementia (FTD) is characterized by apathy, emotional blunting, disinhibition, and impairment in executive functioning despite relatively preserved memory and visuospatial abilities. Cognitive deficits are known to occur in PSP; however, overt clinical FTD without parkinsonism or supranuclear gaze palsy associated with PSP pathology has rarely been documented. We report an elderly patient with the typical clinical, neuropsychometric, and neuroimaging features of FTD who had autopsy findings most consistent with PSP plus AGD and AD in limbic structures. We suggest that PSP with or without coexisting AD and AGD be included in the differential diagnosis of patients presenting with FTD.
进行性核上性麻痹（PSP）的典型表现为垂直性核上性凝视麻痹、病程早期频繁跌倒、轴性强直以及对左旋多巴反应不佳。阿尔茨海默病（AD）的特征是显著的顺行性记忆功能障碍，随后在其他认知领域出现损害。嗜银颗粒病（AGD）尚无明确的临床综合征。额颞叶痴呆（FTD）的特点是冷漠、情感迟钝、行为脱抑制以及执行功能受损，尽管记忆和视觉空间能力相对保留。已知PSP会出现认知缺陷；然而，与PSP病理相关的、无帕金森症或核上性凝视麻痹的明显临床FTD鲜有文献记载。我们报告了一名老年患者，其具有FTD典型的临床、神经心理测量和神经影像学特征，尸检结果显示在边缘结构中最符合PSP加AGD和AD。我们建议，无论是否并存AD和AGD，PSP都应纳入FTD表现患者的鉴别诊断中。
