Semantic dementia combined with motor neuron disease |
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| Authors: | Sook Hui Kim Sang Won Seo Seok Min Go Mee Kyung Suh Juhee Chin Jee Hyang Jeong Duk L. Na |
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| Affiliation: | 1. PET/CT Dementia Research Center, Juntendo Tokyo Koto Geriatric Medical Center, Juntendo University School of Medicine, 3-3-20 Shinsuna, Koto-ku, Tokyo 136-0075, Japan;2. Yokohama Hoyu Hospital, Psychiatry, Yokohama 241-0812, Japan;3. Dementia Research Project, Tokyo Metropolitan Institute of Medical Science, Tokyo 156-8506, Japan;4. Department of Psychiatry, Juntendo University School of Medicine, Tokyo 113-8421, Japan;5. Department of Neurology, Juntendo University School of Medicine, Tokyo 113-8431, Japan;6. Daigo Hospital, Psychogeriatric Center, Miyazaki 889-1911, Japan;1. Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas, Piracicaba, São Paulo, Brazil;2. School of Medical Sciences, University of Campinas, Campinas, São Paulo, Brazil;3. Department of Otorhinolaringology and Head and Neck Surgery, AC Camargo Cancer Center, São Paulo, Brazil;4. Department of Pathology, Dental School, Federal University of Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil;5. Department of Pathology, Dental School, State University of Western Paraná, Cascavel, Paraná, Brazil;6. Department of Oral Pathology and Oral Biology, School of Dentistry, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa;1. Department of Research and Innovation, Reims University Hospital, Reims, France;2. Department of Geriatrics and Internal Medicine, Reims University Hospital, Reims, France;3. Department of Cardiology, Reims University Hospital, Reims, France;4. Regional Centre for Pharmacovigilance and Pharmacoepidemiology, Reims University Hospital, Reims, France;5. Faculty of Medicine, University of Reims Champagne-Ardenne, Reims, France |
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| Abstract: | Up to 20% of patients with behavioural variants of frontotemporal dementia (FTD) also have motor neuron disease (MND); conversely, this comorbidity is rare in patients with language variants of FTD. A few patients have been reported with semantic dementia (SD) combined with MND. However, these patients demonstrated the clinical features of MND in the advanced stage. We report a patient with SD who also demonstrated MND symptoms in an earlier stage of the disease. A 61-year-old man visited our memory disorder clinic as a result of language disturbance and dysarthria of 8 months duration and facial recognition impairment of 3 months duration. Neuropsychological tests revealed anomic aphasia, prosopagnosia, and decreased semantic fluency. A brain MRI revealed significant atrophies localized in both anterior temporal lobes with a greater prominence on the right side. Clinical examination and electrophysiological studies confirmed a diagnosis of MND 17 months after the onset of the disease. |
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