Department of neurology, centre de référence "Démences Rares", hôpital de la Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France; Inserm UMRS 975, CRICM, hôpital de la Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France.
Rev Neurol (Paris). 2013 Oct;169(10):680-6. doi: 10.1016/j.neurol.2013.06.001. Epub 2013 Sep 12.
Language disorders of degenerative origin are frequently tied to Alzheimer disease (AD) the different variants of which can result in primary and secondary aphasia syndromes. More specifically, Alzheimer pathology can primarily erode frontal, temporal or parietal language cortices resulting in three genuine AD language variants which account for about 30% of primary degenerative aphasias. Likewise, it can spread from non-language to language cortices leading to secondary language disorders like in typical amnesic AD and in several atypical AD variants. This paper reviews the whole set of AD variants by characterising their impact on the neural language system and on linguistic functioning. It also provides cues for diagnostic strategies which are essential for linguistic, syndromic and nosological patient classification, for adequate clinical follow-up and for guiding language rehabilitation. Such diagnostic approaches, founded on detailed linguistic phenotyping while integrating anatomical and neuropathological findings, also represent a crucial issue for future drug trials targeting the physio-pathological processes in degenerative aphasias.
退化性语言障碍常与阿尔茨海默病(AD)有关,AD 的不同变体可导致原发性和继发性失语症综合征。更具体地说,阿尔茨海默病病理学可主要侵蚀额、颞或顶叶语言皮质,导致三种真正的 AD 语言变体,占原发性进行性失语症的约 30%。同样,它可以从非语言区域扩散到语言区域,导致继发性语言障碍,如典型的遗忘型 AD 和几种非典型 AD 变体。本文通过描述 AD 变体对神经语言系统和语言功能的影响,综述了整个 AD 变体组。它还为诊断策略提供了线索,这些策略对于语言、综合征和疾病分类的患者、适当的临床随访以及指导语言康复至关重要。这些诊断方法基于详细的语言表型,同时整合解剖和神经病理学发现,对于针对退行性失语症中生理病理过程的未来药物试验也是一个关键问题。