Department of Neuropsychiatry, Ashikaga Red Cross Hospital, Ashikaga-City, Japan.
BMC Neurol. 2013 Nov 1;13:158. doi: 10.1186/1471-2377-13-158.
Due to the nature of neurodegenerative disorders, patients with primary progressive aphasia develop cognitive impairment other than aphasia as the disorder progresses. The progression of logopenic variant primary progressive aphasia (lvPPA), however, has not been well described. In particular, praxic disorders and semantic memory deficits have rarely been reported.
We report three patients in the initial stage of lvPPA who subsequently developed apraxia in the middle stage and developed clinically evident semantic memory deficits in the advanced stages.
The present case series suggests that some patients with lvPPA develop an atypical type of dementia with apraxia and semantic memory deficits, suggesting that these cases should be classified as a type of early-onset Alzheimer's disease.
由于神经退行性疾病的性质，原发性进行性失语症患者随着疾病的进展除了失语症外还会出现认知障碍。然而，流畅性失语变异型原发性进行性失语症（lvPPA）的进展尚未得到很好的描述。特别是，运动障碍和语义记忆缺陷很少有报道。
我们报告了 3 例处于 lvPPA 初始阶段的患者，随后在中期发展为失用症，在晚期发展为明显的语义记忆缺陷。
本病例系列表明，一些 lvPPA 患者会发展为伴有失用症和语义记忆缺陷的非典型痴呆症，这表明这些病例应归类为早发性阿尔茨海默病的一种类型。
