ABSTRACT

Amyotrophic lateral sclerosis (ALS) is a progressive devastating neurological syndrome that produces upper and lower motoneuron loss, manifested as progres­ sive limb and facial motor weakness, atrophy, spasticity and hyperactive reflexes, and eventually respiratory compromise and death. The disorder has currently an incidence of 1-2 per 10,000, which is comparable to that of multiple sclerosis. However, recent epidemiological evidence indicates that the incidence of ALS is increasing in many countries, suggesting a role for environmental factors in the cause of the disease. The disease is present worldwide, but with an increased incidence in regions of the western Pacific, among the Chamorros of Guam, the Auyu and Jakei of West New Guinea, and the Japanese in the Kii peninsula (1).