Semantic dementia combined with motor neuron disease.
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.
Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-dong Kangnam-ku, Seoul, 135-710, Republic of Korea.
Sook Hui
SH
Kim
Sang Won
SW
Seo
Seok Min
SM
Go
Mee Kyung
MK
Suh
Juhee
J
Chin
Jee Hyang
JH
Jeong
Duk L
DL
Na
D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D019788:Fluorodeoxyglucose F18; D057174:Frontotemporal Lobar Degeneration / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000175:diagnosis; D049268:Positron-Emission Tomography / Q000379:methods
D019788:Fluorodeoxyglucose F18