IF 0.6 4区 医学 Q4 CLINICAL NEUROLOGY
Riddhi Patira, Ameneh Zare Shahabadi, Oscar Lopez, Julia Kofler
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引用次数: 0

摘要

非典型额颞叶变性伴泛素阳性包涵体(aFTLD-U)是额颞叶变性的一种罕见亚型。有报道称,aFTLD-U是额颞叶痴呆综合征的一种典型的年轻发病行为变异型,更具体地说,它具有年龄较小、以过度紧张为主、强迫症特征和严重尾状体萎缩等特征。有报道称,极少数病例有突出的语言障碍,但无主要行为特征。我们介绍了另一例罕见的 aFTLD-U 病例,该病例伴有突出的语言障碍,增加了表型谱,其语言障碍主要表现为口语,见于与非流利性原发性进行性失语相关的言语障碍。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
aFTLD-U presenting with primary progressive aphasia suggestive of non-fluent type with apraxia of speech.

Atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U) is a rare subtype of Frontotemporal Lobar Degeneration. aFTLD-U is reported with a typically young onset behavioral variant of frontotemporal dementia syndrome, more specifically with younger age, predominant hyperorality, obsessive-compulsive features, and severe caudate atrophy. Very few cases have been reported with prominent language impairment with no major behavioral features. We present another such rare case of aFTLD-U presenting with prominent language impairment adding to the phenotypic spectrum, with language deficits observed primarily in spoken language seen in apraxia of speech associated with non-fluent primary progressive aphasia.

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来源期刊
Neurocase
Neurocase 医学-精神病学
CiteScore
1.40
自引率
12.50%
发文量
70
审稿时长
6-12 weeks
期刊介绍: Neurocase is a rapid response journal of case studies and innovative group studies in neuropsychology, neuropsychiatry and behavioral neurology that speak to the neural basis of cognition. Four types of manuscript are considered for publication: single case investigations that bear directly on issues of relevance to theoretical issues or brain-behavior relationships; group studies of subjects with brain dysfunction that address issues relevant to the understanding of human cognition; reviews of important topics in the domains of neuropsychology, neuropsychiatry and behavioral neurology; and brief reports (up to 2500 words) that replicate previous reports dealing with issues of considerable significance. Of particular interest are investigations that include precise anatomical localization of lesions or neural activity via imaging or other techniques, as well as studies of patients with neurodegenerative diseases, since these diseases are becoming more common as our population ages. Topic reviews are included in most issues.
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