Typical amyotrophic lateral sclerosis (ALS) is described as a motoneur
on disease which spared cognitive functions. Recent studies reported c
ognitive impairment associated with classical ALS, Gallasi and al. (19
85) detect subtle cognitive impairment sparing memory in a population
of 22 patients affected with sporadic motoneuron disease. Iwasaki and
al. (1990) finds lower scores, including memory tests. Our study evalu
ated 26 patients compared with 26 control subjects with neurophysiolog
ical tests (rapid evaluation of cognitive function fluency Weschler ad
ult intelligence scale, Wisconsin cards, Rey scheme, memory tests - Lu
ria -, trail mailing, visual retentional test of Benton Violon Seyll t
est). All the neuropsychological tests were significantly lower for th
e patients group. The cognitive impairment is global: memory and front
al functions were not spared and this impairment is also subtle. It ma
y easily go undetected without tests. We cannot isolate a cortical or
subcortical profile of the deterioration.