Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology

Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology
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DOI:
10.1159/000068502
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发表时间:
2003-01-01
期刊:
影响因子:
2.4
通讯作者:
Petersen, R
Petersen, R
中科院分区:
医学4区
文献类型:
--
作者:
Boeve, B;Dickson, D;Petersen, R

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我们描述了一位右撇子,他从 71 岁开始出现进行性失语症和言语失用症。到 74 岁时,他出现行为改变、失语性痴呆以及轻度帕金森症;除了向上凝视轻度受限外,眼外运动正常。系列神经心理测试和单光子发射计算机断层扫描(SPECT)扫描显示进行性变化,反映左>右大脑半球功能障碍。神经病理学检查显示进行性核上性麻痹 (PSP) 的特征,但皮质病理学比典型的 PSP 更广泛。我们的结论是,该病例的临床表现更类似于进行性非流利性失语症并随后出现失语性痴呆和轻度帕金森症,而不是 PSP。因此,PSP在临床上可以表现为一种以进行性失语/失用为主的非典型痴呆综合征。版权所有 (C) 2003 S. Karger AG,巴塞尔。
We describe a right-handed man who developed progressive nonfluent aphasia and apraxia of speech beginning at age 71. By age 74 he had behavioral changes, aphasic dementia, as well as mild parkinsonism; extraocular movements were normal except for mild limitation of upgaze. Serial neuropsychometric testing and single photon emission computed tomography (SPECT) scans showed progressive changes reflecting left>right cerebral hemisphere dysfunction. Neuropathologic examination revealed findings characteristic of progressive supranuclear palsy (PSP) except that the cortical pathology was more widespread than is typical of PSP. We conclude that the clinical manifestations in this case were more similar to the syndrome of progressive nonfluent aphasia with subsequent aphasic dementia and mild parkinsonism, rather than those of PSP. Hence, PSP can present clinically as an atypical dementing syndrome dominated by progressive aphasia/apraxia of speech. Copyright (C) 2003 S. Karger AG, Basel.