Prominent auditory deficits in primary progressive aphasia: A case study.
Prominent auditory deficits in primary progressive aphasia: A case study.
复制标题
原发性进行性失语症的显着听觉缺陷：案例研究。
DOI：
10.1016/j.cortex.2019.01.021
复制
发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Josephs,KeithA
中科院分区：
文献类型：
--
作者：
Utianski,ReneL;Duffy,JosephR;Clark,HeatherM;Machulda,MaryM;Dickson,DennisW;Whitwell,JenniferL;Josephs,KeithA
失语症通常与语言表达和理解的书面和口头形式的相当困难有关。相比之下，听觉言语失认症是在没有初级感觉缺陷的情况下，与理解书面语言相比，理解口头语言的困难不成比例，通常也比口语和书面语言表达的困难更大。纯文字失聪和听觉言语失认症通常是同义词。然而，广义的“听觉失认症”更准确地反映了语音和非语音处理的困难，而患有听觉言语失认症（纯粹的文字失聪）的个体则保留了对环境声音的处理。听觉失认症在中风文献中有报道，但在进行性神经系统疾病中很少报道。在这里，我们报告了一例女性，她在最初无法分类或混合的原发性进行性失语症（PPA）伴语言失用的背景下表现出明显的听觉缺陷。她的临床表现与听觉失认症有共同的特征，尽管感觉功能没有正式评估。我们报告临床和神经影像学数据跨越6年和随后的尸检结果。她在65岁时出现症状，发病5年后出现症状，包括潜在的和进行性的单词思考困难、造句困难、发音困难和理解说明困难。与理解书面语言相比，她在理解口语方面有不成比例的困难。她最终发展出PPA的不流利/语法变体的特征，以及言语失用症。[18F]-氟脱氧葡萄糖(FDG)-PET成像显示双侧（左大于右）代谢降低累及额叶、颞叶（主要是外侧上回）和顶叶，最终包括辅助运动区、前扣带和尾状核。尸检显示病理病变符合皮质基底变性。
Aphasia typically is associated with comparable difficulties in written and spoken modalities of language expression and comprehension. In contrast, auditory verbal agnosia is the disproportionate difficulty comprehending spoken compared to written language, also typically greater than difficulties with spoken and written language expression, in the absence of a primary sensory deficit. The terms pure word deafness and auditory verbal agnosia are often used synonymously. However, the broader term of auditory agnosia more accurately reflects difficulty processing both speech and non-speech sounds whereas individuals with auditory verbal agnosia (pure word deafness) have preserved processing of environmental sounds. Auditory agnosia is reported in the stroke literature, but rarely reported in progressive neurologic disorders. Here, we report a case of a woman who presented with what is best described as a prominent auditory deficit in the context of an initially unclassifiable, or mixed, primary progressive aphasia (PPA) with accompanying apraxia of speech. Her clinical presentation shared features with auditory agnosia, although sensory functioning was not formally assessed. We report clinical and neuroimaging data spanning 6 years and subsequent autopsy results. She presented at 65 years of age, 5 years post onset of symptoms that included insidious and progressive difficulties thinking of words, constructing sentences, pronouncing words, and understanding instructions. She had disproportionate difficulty with comprehension of spoken compared to written language. She eventually developed features of the nonfluent/agrammatic variant of PPA, as well as an apraxia of speech. Imaging with [18F]-fluorodeoxyglucose (FDG)-PET revealed progression of bilateral (left greater than right) hypometabolism involving the frontal, temporal (predominantly the lateral superior gyrus), and parietal lobes, that eventually included the supplementary motor area, anterior cingulate, and caudate. Autopsy revealed pathological lesions consistent with corticobasal degeneration.