AMYOTROPHIC-LATERAL-SCLEROSIS WITH DEMENTIA

Four patients with amyotrophic lateral sclerosis (ALS) and dementia ar e reported. Mental symptoms antedated motor signs and were investigate d with a neuropsychology battery, which brought out different patterns of cognitive impairment. A case presented with frontal dementia, anot her with a predominant aphasic, apraxic, amnesic syndrome, while the r emainders showed cognitive decline in association with blunt affect. M otor signs were characterized by a precocious involvement of the upper motor system.