This is the second paper of a series of four on recent investigations of the amyotrophic lateral sclerosis/parkinsonism-dementia (ALS/P-D) complex on the island of Guam.
The objectives of this report are as follows: (1) to analyze in detail the neurological features of Guam ALS and P-D and compare them with classical ALS, Parkinson's disease, and other relevant disorders: (2) to show the unique relationship between the ALS and P-D syndromes, clinically and histologically; and (3) to report on pertinent laboratory studies.
The data of this study were collected over a period of seven years (1957-1964). Related epidemiologic, neuropathologic, and genetic studies will be reported in separate papers.
Historical Background
The unusual prevalence of a "hereditary paralysis" on Guam was first reported in the medical literature in 1900.1 The diagnosis of "progressive muscular atrophy" was recorded on local death certificates as early as 1904, and the diagnosis of "amyotrophic lateral