Molecular neuroimaging in primary progressive aphasia with predominant agraphia.
Molecular neuroimaging in primary progressive aphasia with predominant agraphia.
复制标题
原发性进行性失语伴显性失写症的分子神经影像学。
DOI：
10.1080/13554794.2018.1454963
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发表时间：
2018
期刊：
影响因子：
0.8
通讯作者：
Josephs,KeithA
中科院分区：
文献类型：
--
作者：
Utianski,ReneL;Duffy,JosephR;Savica,Rodolfo;Whitwell,JenniferL;Machulda,MaryM;Josephs,KeithA
一名 62 岁男性出现进行性孤立书写和拼写困难。神经学、神经心理学、言语和语言评估仅发现了极少的额外异常。所呈现的特征不符合原发性进行性失语症任何特定变体的标准；他的临床表现最恰当的描述是原发性进行性失语症，主要表现为几乎纯粹的失写症。脑部 MRI 显示不对称的双侧脑实质体积减少，左侧海马萎缩。氟脱氧葡萄糖-F18正电子发射断层扫描显示左额叶外侧代谢低下，包括埃克斯纳区。 β-淀粉样蛋白和 tau-正电子发射断层扫描呈阴性，表明病因不是阿尔茨海默病。尽管不能排除 4 次重复的 tau 蛋白病，但潜在的神经退行性过程很可能与 TDP-43 有关。跟随他的临床演变，并最终从尸检中确定潜在的病理学，将阐明这种有趣的临床表现的病因。
A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy. Fluorodeoxyglucose-F18 positron emission tomography showed hypometabolism in the lateral left frontal lobe, including Exner’s area. Beta-amyloid and tau-positron emission tomography scans were negative, indicating the etiology was not Alzheimer’s disease. The underlying neurodegenerative process is most likely related to TDP-43, although a 4-repeat tauopathy cannot be excluded. Following his clinical evolution, and ultimately identifying the underlying pathology from autopsy, will elucidate the etiology of this interesting clinical presentation.