尽管最初低估了皮质基底综合征(CBS)的语言功能障碍,失语症现在被认为是这种疾病的常见特征。CBS中的失语症似乎在临床上覆盖非流利/农原性原发性进行性失语症(nfaPPA),这也是与皮质基底变性(CBD)病理相关的临床表型。然而,CBS失语症的临床特征仍未得到很好的描述,导致疾病的PPA表现在鉴别诊断中的误判。
为了调查这种综合征的语言障碍,还通过系统检查重新编码技能(阅读,书面拼写和重复)和发音障碍,这在以前的研究中很少被检查。
我们对12例失语症患者的语言障碍进行了临床和神经心理学描述性研究。语言评估是通过EsameNeuroPsicologicodell\'Afasia进行的,一个全面的意大利语电池,用于语言功能,令牌测试,和言语失用症评定量表。
患者的语言特点显示有严重的表达性语言障碍,以非流利的语言为特征,言语失用症(AoS),主要表现为类似口吃的言语障碍,空间/失写症,缺乏单词查找和有缺陷的句子重复。严重肢体失用症,还存在视觉空间缺陷和异形手综合征。神经影像学显示双侧左不对称萎缩和前额运动前代谢减退,顶叶后部和颞区。
这些发现表明,CBS中的失语症可能表现为“混合PPA”,而不是如前所述的nfaPPA,显示PPA的nfa和logopenic变体的特征组合,与AoS有关,口吃和失写症,这可能是CBS临床诊断和区分CBDnfaPPA表现特征的其他重要认知标记。这些结果也可能表明CBS患者康复的具体干预领域。本文补充了CBS患者的语言障碍通常在临床上覆盖nfaPPA,这也是与CBD病理学相关的临床表型,根据最近的诊断标准。然而,CBS失语症的临床特征仍未得到很好的描述,这给临床医生从疾病的PPA表现进行鉴别诊断带来了困难和误判。本文对现有知识的补充这项研究表明,我们的CBS患者的语言特点是严重的表达性语言障碍,说话不流利,言语失用症(AoS),主要表现为类似口吃的言语障碍,空间/失写症,缺乏单词查找,有缺陷的句子重复。这些发现表明,CBS中的失语症可能表现为“混合PPA”,而不是如前所述的nfaPPA,显示与AoS相关的PPA的nfa和logopenic变体的特征组合,口吃和失写症。这项工作的潜在或实际临床意义是什么?这些结果表明,AoS,口吃和失写可能是CBS临床诊断的重要额外认知标志物,和区分CBD的nfaPPA表示的特征。本研究中表现出的语言障碍也可能支持言语和语言治疗师针对CBS患者康复中的特定干预领域。
Despite initial underreporting of language dysfunctions in corticobasal syndrome (CBS), aphasia is now recognized as a frequent feature of this disease. Aphasia in CBS seems clinically overlying to a non-fluent/agrammatic primary progressive aphasia (nfaPPA), which is also a clinical phenotype associated with corticobasal degeneration (CBD) pathology. However, the clinical features of aphasia in CBS still remain poorly delineated, resulting in misjudgements in the differential diagnosis from a PPA presentation of the disease.
To investigate the language disorders of this syndrome, also through a systematic examination of recoding skills (reading, written spelling and repetition) and articulatory disturbances, which have been rarely examined in previous studies.
We present a clinical and neuropsychological descriptive study of the language impairments in a
case series of 12 aphasic patients with a clinical diagnosis of CBS. Language assessment was conducted by means of the Esame NeuroPsicologico dell\'Afasia, a comprehensive Italian battery for language functions, the Token Test, and the Apraxia of Speech Rating Scale.
The language profile of the patients showed a severe expressive language disorder, characterized by non-fluent speech, apraxia of speech (AoS) with predominant stuttering-like dysfluencies, spatial/apraxic agraphia, lack of word-finding and defective sentence repetition. Severe limb apraxia, visual-spatial deficit and alien hand syndrome were also present. Neuroimaging showed bilateral left asymmetric atrophies and hypometabolism in the frontal premotor, parietal posterior and temporal areas.
These findings suggest that aphasia in CBS might present as a \'mixed PPA\', instead of an nfaPPA as previously stated, showing a combination of features of the nfa and logopenic variants of the PPA, associated with AoS, stuttering and agraphia, which might be additional important cognitive markers for the clinical diagnosis of CBS and discriminating features of an nfaPPA presentation of a CBD. These results might also suggest specific intervention areas in the rehabilitation of patients with CBS. What this paper adds What is already known on the subject Language disorders in CBS patients usually present clinically overlying to an nfaPPA, which is also a clinical phenotype associated with CBD pathology, according to recent diagnostic criteria. However, the clinical features of aphasia in CBS still remain poorly delineated, and this raises difficulties and misjudgements for clinicians in the differential diagnosis from a PPA presentation of the disease. What this paper adds to existing knowledge This study shows that the language profile of our CBS patients was characterized by severe expressive language disorders, with non-fluent speech, apraxia of speech (AoS) with predominant stuttering-like dysfluencies, spatial/apraxic agraphia, lack of word-finding, and defective sentence repetition. These findings suggest that aphasia in CBS might present as a \'mixed PPA\', rather than an nfaPPA as previously stated, showing a combination of features of the nfa and logopenic variants of the PPA associated with AoS, stuttering and agraphia. What are the potential or actual clinical implications of this work? These results suggest that AoS, stuttering and agraphia might be important additional cognitive markers for the clinical diagnosis of CBS, and discriminating features of an nfaPPA presentation of a CBD. The language disorders exhibited in the present study might also support speech and language therapists in targeting specific intervention areas in the rehabilitation of patients with CBS.