Clinical, radiologic, and pathologic features of the globular glial tauopathy subtype of frontotemporal lobar degeneration in right temporal variant frontotemporal dementia with salient features of Geschwind syndrome.

IF 0.6 4区医学 Q4 CLINICAL NEUROLOGY

Neurocase Pub Date : 2022-08-01 Epub Date: 2022-10-17 DOI:10.1080/13554794.2022.2130805

Sylvia Josephy-Hernandez, Michael Brickhouse, Samantha Champion, David Dongkyung Kim, Alexandra Touroutoglou, Matthew Frosch, Bradford C Dickerson

引用次数: 0

Abstract

Globular Glial Tauopathy (GGT) is a rare form of Frontotemporal Lobar Degeneration (FTLD) consisting of 4-repeat tau globular inclusions in astrocytes and oligodendrocytes. We present the pathological findings of GGT in a previously published case of a 73-year-old woman with behavioral symptoms concerning for right temporal variant frontotemporal dementia with initial and salient features of Geschwind syndrome. Clinically, she lacked motor abnormalities otherwise common in previously published GGT cases. Brain MRI showed focal right anterior temporal atrophy (indistinguishable from five FTLD-TDP cases) and subtle ipsilateral white matter signal abnormalities. Brain autopsy showed GGT type III and Alzheimer's neuropathologic changes. .

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具有Geschwind综合征显著特征的右颞变异性额颞叶痴呆的额颞叶变性球状神经胶质tau病亚型的临床、放射学和病理特征。

球状神经胶质tau病（GGT）是一种罕见的额颞叶退行性变（FTLD），由星形胶质细胞和少突胶质细胞中的4个重复tau球状内含物组成。我们介绍了一例先前发表的GGT的病理结果，该病例为一名73岁女性，其行为症状与右颞变异额颞叶痴呆有关，具有Geschwind综合征的初始和显著特征。在临床上，她没有运动异常，这在之前发表的GGT病例中很常见。脑MRI显示局灶性右前颞叶萎缩（与5例FTLD-TDP病例无法区分）和细微的同侧白质信号异常。尸检显示GGT III型和阿尔茨海默氏症的神经病理改变。 .

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来源期刊

Neurocase 医学-精神病学

CiteScore

1.40

自引率

12.50%

发文量

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.