Departments of Neurology.
Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Alzheimer Dis Assoc Disord. 2022;36(4):365-367. doi: 10.1097/WAD.0000000000000498. Epub 2022 Mar 15.
Primary progressive apraxia of speech (PPAOS), a rare neurodedegenerative disorder, can be subdivided into predominant phonetic or prosodic type. Pure prosodic type of PPAOS as an isolated disorder has been hardly found. We present 2 cases of patients with pure prosodic PPAOS who initially were misdiagnosed as nonfluent variant of primary progressive aphasia and later turned out to be corticobasal syndrome. A 65-year-old woman and a 72-year-old man were referred to our speech-language clinic under the clinical impression of nonfluent variant of primary progressive aphasia. The neurological examinations revealed no definite abnormalities except for slow and effortful speech with the production of simple sentences. However, their receptive and expressive language abilities were normal. Their brain magnetic resonance imaging was unremarkable. We initially entertained the diagnosis of pure prosodic type of PPAOS. During several years of follow up, they gradually developed extrapyramidal symptoms which are compatible with corticobasal syndrome. The characteristics of the patients and the results of neuroimaging studies are discussed.
原发性进行性构音障碍（PPAOS）是一种罕见的神经退行性疾病，可分为以语音为主或韵律为主的类型。纯粹的韵律型 PPAOS 作为一种孤立的疾病很少被发现。我们报告了 2 例纯粹韵律型 PPAOS 患者，他们最初被误诊为非流利型原发性进行性失语，后来被诊断为皮质基底节综合征。一名 65 岁女性和一名 72 岁男性因非流利型原发性进行性失语的临床印象被转介到我们的言语语言诊所。神经系统检查除了简单句子的产生出现缓慢而费力的言语外，没有明显的异常。然而，他们的接受性和表达性语言能力正常。他们的脑部磁共振成像无明显异常。我们最初考虑了纯粹韵律型 PPAOS 的诊断。在几年的随访中，他们逐渐出现了与皮质基底节综合征相符的锥体外系症状。讨论了患者的特征和神经影像学研究的结果。
