ALS-like disorders of the Western Pacific are also known as or subsume Amyotrophic lateral sclerosis-parkinsonism-dementia complex, Guam neurodegeneration, Lytico-bodig, and Marianas dementia. -ed.
Following World War II, extremely high rates of a neurodegenerative disease with features of amyotrophic lateral sclerosis (ALS), dementia, and Parkinson disease were described in the Western Pacific islands of Guam, the Kii peninsula of Japan, and West Papua, Indonesia. Termed lytico-bodig by natives, amyotrophic lateral sclerosis-parkinsonism-dementia complex (ALS-PDC) has distinct neuropathologic features of widely distributed neurofibrillary tangles comprised of tau in addition to pathologic findings of amyotrophic lateral sclerosis. Incidence rates have declined dramatically over subsequent decades, with a change in phenotype to predominantly dementia. The etiology of ALS-PDC is most likely a combination of environmental agents coupled with genetic susceptibility.