{"title":"原发性脑损伤伴语意缺失/语义混合进行性失语和额颞叶痴呆样行为。","authors":"Akihiro Takasaki, Yoshiyuki Nishio, Yuto Satake, Matasaburo Kobayashi, Mariko Sakai, Kohji Mori, Kazunari Ishii, Manabu Ikeda","doi":"10.1097/WNN.0000000000000391","DOIUrl":null,"url":null,"abstract":"<p><p>Here we present the cases of two individuals with language and behavioral symptoms indicative of the early clinical manifestations of both Alzheimer disease (AD) and frontotemporal lobar degeneration (FTLD). Phonological language symptoms similar to those evident in logopenic variant primary progressive aphasia suggested AD pathology, while semantic impairment and behavioral changes (ie, abnormal eating behavior and disinhibition) suggested a diagnosis of FTLD. Multimodal neuroimaging studies revealed underlying neuropathology indicative of primary tauopathy with presumable 3/4-repeat isoform, devoid of amyloid deposition. We suggest that these cases may represent a previously unrecognized syndrome associated with non-Alzheimer primary tauopathy.</p>","PeriodicalId":50671,"journal":{"name":"Cognitive and Behavioral Neurology","volume":" ","pages":""},"PeriodicalIF":1.3000,"publicationDate":"2025-04-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Primary Tauopathy With Logopenic/Semantic Mixed Progressive Aphasia and Frontotemporal Dementia-like Behavior.\",\"authors\":\"Akihiro Takasaki, Yoshiyuki Nishio, Yuto Satake, Matasaburo Kobayashi, Mariko Sakai, Kohji Mori, Kazunari Ishii, Manabu Ikeda\",\"doi\":\"10.1097/WNN.0000000000000391\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Here we present the cases of two individuals with language and behavioral symptoms indicative of the early clinical manifestations of both Alzheimer disease (AD) and frontotemporal lobar degeneration (FTLD). Phonological language symptoms similar to those evident in logopenic variant primary progressive aphasia suggested AD pathology, while semantic impairment and behavioral changes (ie, abnormal eating behavior and disinhibition) suggested a diagnosis of FTLD. Multimodal neuroimaging studies revealed underlying neuropathology indicative of primary tauopathy with presumable 3/4-repeat isoform, devoid of amyloid deposition. We suggest that these cases may represent a previously unrecognized syndrome associated with non-Alzheimer primary tauopathy.</p>\",\"PeriodicalId\":50671,\"journal\":{\"name\":\"Cognitive and Behavioral Neurology\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":1.3000,\"publicationDate\":\"2025-04-18\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cognitive and Behavioral Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1097/WNN.0000000000000391\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"BEHAVIORAL SCIENCES\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cognitive and Behavioral Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/WNN.0000000000000391","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"BEHAVIORAL SCIENCES","Score":null,"Total":0}
Primary Tauopathy With Logopenic/Semantic Mixed Progressive Aphasia and Frontotemporal Dementia-like Behavior.
Here we present the cases of two individuals with language and behavioral symptoms indicative of the early clinical manifestations of both Alzheimer disease (AD) and frontotemporal lobar degeneration (FTLD). Phonological language symptoms similar to those evident in logopenic variant primary progressive aphasia suggested AD pathology, while semantic impairment and behavioral changes (ie, abnormal eating behavior and disinhibition) suggested a diagnosis of FTLD. Multimodal neuroimaging studies revealed underlying neuropathology indicative of primary tauopathy with presumable 3/4-repeat isoform, devoid of amyloid deposition. We suggest that these cases may represent a previously unrecognized syndrome associated with non-Alzheimer primary tauopathy.
期刊介绍：
Cognitive and Behavioral Neurology (CBN) is a forum for advances in the neurologic understanding and possible treatment of human disorders that affect thinking, learning, memory, communication, and behavior. As an incubator for innovations in these fields, CBN helps transform theory into practice. The journal serves clinical research, patient care, education, and professional advancement.
The journal welcomes contributions from neurology, cognitive neuroscience, neuropsychology, neuropsychiatry, and other relevant fields. The editors particularly encourage review articles (including reviews of clinical practice), experimental and observational case reports, instructional articles for interested students and professionals in other fields, and innovative articles that do not fit neatly into any category. Also welcome are therapeutic trials and other experimental and observational studies, brief reports, first-person accounts of neurologic experiences, position papers, hypotheses, opinion papers, commentaries, historical perspectives, and book reviews.
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