Amyotrophic lateral sclerosis (ALS) occupies a unique place in the history of human disease in general and in neurological disease in particular. Charcot was the one who deduced the relationship between the clinical signs and the findings at autopsy. In his 1874 description,1 Charcot established the clinicopathologic approach that has dominated medical nosology ever since. In the latter half of the 19th century, diseases were defined by autopsy findings.