シンポジウム07―4 Atypical types of dementia非典型認知症 Dementia with motor neuron disease

The neurodegenerative process of amyotrophic lateral sclerosis (ALS) includes both selective degeneration of motor neurons and one or more syndromes of frontotemporal dysfunction (e.g., behavioural (ALSbi) or cognitive(ALSci) syndromes, a frontotemporal dementia(ALS-FTD)) .There is widespread degeneration of the frontal, temporal and to a lesser extent parietal lobes in association with TDP-43 immunoreactive pathology. We have shown that tau protein metabolism is also altered in ALSci and now describe the topographic distribution of tau pathology in ALS.