Article

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of
motor neurons, resulting in worsening weakness of voluntary muscles until death
occurs from respiratory failure. The incidence of ALS in European populations is
two to three people per year per 100,000 of the general population. In Europe,
crude prevalences range from 1.1/100,000 population in Yugoslavia to 8.2/100,000
in the Faroe Islands. Major advances have been made in our understanding of the
genetic causes of ALS, whereas the contribution of environmental factors has been
more difficult to assess and large-scale studies have not yet revealed a
replicable, definitive environmental risk factor.
The only established risk
factors to date are older age, male gender and a family history of ALS. Median
survival time from onset to death is usually 3 years from the first appearance of
symptoms. Older age and bulbar onset are consistently reported to have poorer
outcomes. However, there are conflicting data regarding gender, diagnostic delay
and El Escorial criteria. The rate of symptom progression has been revealed to be
an independent prognostic factor. Psychosocial factors and impaired cognitive
function are negatively related to ALS outcome, while nutritional status and
respiratory function are also related to ALS prognosis.
The effect of enteral
nutrition on survival is still unclear, although noninvasive positive pressure
ventilation (NIPPV) has been found to improve survival.
These findings have
relevant implications for the design of future trials.
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