Amyotrophic lateral sclerosis among the migrant population to Piemonte, northwestern Italy.

Recent surveys indicate a decreasing north-to-south gradient in the mortality and incidence rates of amyotrophic lateral sclerosis (ALS) in Italy, possibly indicating a different susceptibility to ALS in these populations. Piemonte, a region of northwestern Italy, experienced a considerable migration from other regions in Italy between 1940 and 1975; we therefore analyzed the effects of place of birth and migration upon the risk of developing ALS. Data on all ALS cases occurring in Piemonte during the period 1971-1990 were collected. Standardized incidence ratios (SIRs) for patients born outside Piemonte were calculated, using the Piemonte-born population as reference. A total of 962 ALS cases were identified during the study period, corresponding to a mean annual crude incidence rate of 1.37/100,000 population (95% confidence interval, 1.29-1.46). The SIRs of patients born in three southern Italian regions and of foreign-born persons were significantly higher than those of persons born in Piemonte and or other regions in Italy, and increased with age. This observation may be explained by an interaction between environmental and genetic factors or by selective migration.

Reduced glutathione in amyotrophic lateral sclerosis: an open, crossover, randomized trial.

The present study set out to define the possible effect of reduced glutathione (GSH), the substrate of glutathione peroxidase (GSH-Px), a free radical inactivating enzyme, in amyotrophic lateral sclerosis (ALS). Thirty-two patients affected by definite ALS seen in our institution between August 1993 and July 1994 were admitted to the study. The effect of GSH was studied in an open, crossover, randomized study. GSH was given at the dose of 600 mg each day intramuscularly for 12 weeks. The patients, taken sequentially, were randomly assigned to two groups. The first group received the drug while the second received only symptomatic therapies for 12 weeks. After a week of washout, the second group received GSH and the first only symptomatic therapies for 12 weeks. The rate of progression of the diseases was compared in the two groups. Clinical evaluation included manual test for muscle strength, Norris scale, bulbar scale, and forced vital capacity (FVC) percent. No significant difference was found in the progression of ALS in the two periods, although a slight slowing of the disease progression rate was found during the period of treatment, probably related to the open design of the study. Our data do not show any significant effect of reduced glutathione in modifying the progression of ALS.