George Annamma, Mathuranath P S
Cognition and Behavioural Neurology Center, Department of Neurology, SCTIMST, Trivandrum, Kerala, India.
Neurol India. 2005 Jun;53(2):162-5; discussion 165-6. doi: 10.4103/0028-3886.16398.
Primary progressive aphasia (PPA), a degenerative disorder, is often misdiagnosed as Alzheimer's disease. Its subtypes, semantic dementia (SD), and progressive nonfluent aphasia (PNFA), are often difficult to differentiate from each other. Our objective was to highlight the differences in the language profiles of patients with SD and PNFA. To bring out these differences, we report two patients with PPA, one with SD and the other with PNFA. They were administered the Western aphasia battery (WAB) and a semantic battery, which assesses semantic memory. The profiles of language impairment on the WAB indicated that the patient with PNFA had syntactic errors in expressive speech but relatively preserved semantics and comprehension, whereas the patient with SD had preserved syntax but made semantic errors in expressive speech, and had impaired comprehension. There were differences in their performance on the semantic battery too. The patient with SD made relatively less errors on confrontation naming, although on the pointing task he failed to point to those line drawings, which he was unable to name on confrontation. In contrast, the finding of the PNFA patient was the reverse of this. Supplementing conventional neuropsychological tests with formal tests for assessment of language functions is useful in the early diagnosis of PPA. The performance of PPA patients on a detailed assessment of language that includes use of formal tests such as the semantic battery helps to differentiate PNFA from SD.
原发性进行性失语（PPA）是一种退行性疾病，常被误诊为阿尔茨海默病。其亚型，语义性痴呆（SD）和进行性非流利性失语（PNFA），往往难以相互区分。我们的目的是突出SD和PNFA患者语言特征的差异。为了揭示这些差异，我们报告了两名PPA患者，一名患有SD，另一名患有PNFA。他们接受了西方失语成套测验（WAB）和一个评估语义记忆的语义成套测验。WAB上的语言损伤特征表明，PNFA患者在表达性言语中有句法错误，但语义和理解相对保留，而SD患者句法保留，但在表达性言语中有语义错误，且理解受损。他们在语义成套测验上的表现也存在差异。SD患者在对名称任务中犯的错误相对较少，尽管在指认任务中，他未能指向那些他在对名称任务中无法说出名称的线条图。相比之下，PNFA患者的情况则相反。用正式的语言功能测试补充传统的神经心理学测试，对PPA的早期诊断很有用。PPA患者在包括使用语义成套测验等正式测试的详细语言评估中的表现有助于区分PNFA和SD。
