β-N-Methylamino- L -alanine (BMAA) has been
linked to Guam ALS/PDC and shown to produce neurodegen-
eration in vitro and in vivo (Drosophila, mice, rats, primates).
BMAA misincorporation into neuroproteins produces protein
misfolding and is inhibited by L -serine. Case-control studies in
Northern New England indicate that living near to water-
bodies with cyanobacterial blooms increases the risk of devel-
oping amyotrophic lateral sclerosis (ALS). The distribution of
addresses of ALS cases in New Hampshire, Vermont, and
Florida was compared to that of controls. Areas of statistically
significantly increased numbers of ALS cases were examined
for sources of environmental toxins. A phase I trial of oral L -
serine was performed in 20 ALS patients (0.5 to 15 g twice
daily). Safety and tolerability were assessed by comparing the
rate of deterioration with 430 matched placebo controls. The
distribution of residential addresses of ALS cases in New
England and Florida revealed many areas where the age-
and gender-adjusted frequency of ALS was greater than ex-
pected (P < 0.01). GIS studies of these Bhot spots^ in relation
to sources of environmental pollutants, like cyanobacterial
blooms, Superfund and Brownfield sites, and landfills, are

EB00000002596K

Available

• Studies of Environmental Risk Factors in Amyotrophic Lateral Sclerosis (ALS) and a Phase I Clinical Trial of L -Serine