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Other cognitive domains were impaired, yet 2 patients lived alone until 1 month before their deaths. Four died within 2 years. Abnormalities were found on electromyography, computed tomography, magnetic resonance imaging, single\u2010photon emission computed tomography, and electroencephalography. Neuropathological examination in 3 patients showed bilateral hemispheric atrophy with neuronal loss and gliosis predominantly of superficial cortical layers. Pigmented and hypoglossal nuclei were relatively preserved. At all spinal levels there was degeneration of corticospinal tracts and loss of anterior horn cells with gliosis. Rapidly progressive aphasic dementia and motor neuron disease are a distinctive clinical entity whose nosology is poorly understood.<\/jats:p>","DOI":"10.1002\/ana.410330210","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T14:33:24Z","timestamp":1104590004000},"page":"200-207","source":"Crossref","is-referenced-by-count":159,"title":["Rapidly progressive aphasic dementia and motor neuron disease"],"prefix":"10.1002","volume":"33","author":[{"given":"Richard J.","family":"Caselli","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anthony J.","family":"Windebank","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald 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