Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex of Guam

Abstract

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