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A Case Report of Frontotemporal Dementia with Motor Neuron Disease Yoshio Mitsuyama 1 , Yoshimasa Ninomiya 2 , Hisdetoshi Ushiro 2 1Dementia Center, Daigo Hospital, Miyazaki, Japan 2Dementia Center, Kyowa Hospital Keyword: 前頭側頭型認知症 , frontotemporal dementia , FTD , 運動ニューロン疾患 , motor neuron disease , MND pp.261-268
Published Date 2021/2/15
DOI https://doi.org/10.11477/mf.1405206283
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 We report a 54-year-old man with frontotemporal dementia with motor neuron disease(FTD-MND). From the second half after 49, he developed psychoneurotic problems characterized with disinhibited behaviors with personality change and difficulty of dysfunction of spontaneous speech such as echolalia and “stehende Redensarten”. General cognitive functions were progressively deteriorated. Head CT-scan revealed atrophy of the bilateral frontotemporal regions that made the clinical diagnosis of frontotemporal dementia. The clinical course showed constantly worsening and he was unable to work in one year later. Three years after the onset, study of the cerebrospinal fluid was normal value as phosphrylated tau and amyloid βproteins. The electroencephalogram showed borderline records with generalized slowing without paroxysmal patterns. Three and a half years later of the onset of his illness, progressive atrophy of the selective distal upper limb muscles as thenar, hypothenar and tongue(lower motor neuron involvement:MND) were identified. While the other limb muscles were spared and preserved the ability to walk even at advanced stage of the disease. Rapid body weight loss(from56.1 to 46.0 in 4 months) was observed. He decreased one year after the onset of MND. The clinical course after the onset of MND was different from that of usual cases of FTD-MND. This case could be regarded as a manifestation of wide etiopathogenesis of FTD-MND.


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電子版ISSN 1882-126X 印刷版ISSN 0488-1281 医学書院

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