Chen Kai-Hsiang, Chiu Ming-Jang, Cheng Ting-Wen, Su Jen-Jen
Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan.
Acta Neurol Taiwan. 2011 Sep;20(3):202-8.
Case reports and a review of literature of the coexistence of motor neuron disease (MND) and frontotemporal dementia (FTD).
All three patients demonstrated generalized lower motor neuron signs and very few upper motor neuron signs. In the level of patterns of cognitive impairments, neuropsychological studies do not distinguish between patients with onset of weakness from bulbar palsy and patients with onset of weakness from limbs. All patients of FTD had their onset of MND or amyotrophic lateral sclerosis symptoms within a two-year interval which is similar to previous reports. FTD combined with MND may shorten the survival to less than three years shorter than cases with FTD only. Respiratory failure occurred one to two years after onset of the behavioral symptoms in all patients.
We reported three patients of FTD with MND to remind clinicians that these two disorders may occur together on the same patient and that these two disorders may belong to one broad spectrum neurodegenerative disease.
motor neuron disease, amyotrophic lateral sclerosis, frontotemporal dementia, frontotemporal lobar degeneration.
运动神经元病（MND）与额颞叶痴呆（FTD）共存的病例报告及文献综述。
所有三名患者均表现出全身性下运动神经元体征，而上运动神经元体征极少。在认知障碍模式层面，神经心理学研究无法区分延髓麻痹导致肌无力起病的患者和肢体肌无力起病的患者。所有FTD患者在两年内出现MND或肌萎缩侧索硬化症状，这与既往报告相似。FTD合并MND可能会使生存期缩短至不到三年，比仅患有FTD的病例短。所有患者在行为症状出现后的一到两年内发生呼吸衰竭。
我们报告了三例FTD合并MND的患者，以提醒临床医生这两种疾病可能在同一患者身上同时发生，且这两种疾病可能属于一种广泛的神经退行性疾病。
运动神经元病；肌萎缩侧索硬化；额颞叶痴呆；额颞叶变性
