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April 1967

Amyotrophic Lateral Sclerosis: A Comparison of Guam and Classical Cases

Author Affiliations

New York
From the Henry and Lucy Moses Research Laboratories of the Laboratory Division, Montefiore Hospital and Medical Center, Bronx, New York. Dr. Arumugasamy is from the Ministry of Health, Malaysia. He is a resident in neurosurgery, Wesley Memorial Hospital, Chicago, assigned to the Neuropathology Department, Laboratory Division, Montefiore Hospital and Medical Center.

Arch Neurol. 1967;16(4):357-363. doi:10.1001/archneur.1967.00470220021003

Amyotrophic lateral sclerosis (ALS), a disease well known for almost 100 years, occurs throughout the world. Approximately 15 years ago, an unusually high incidence of ALS was noted among the indigenous Chamorro population on Guam of the Mariana Islands. Subsequently, detailed epidemiological, clinical, and pathological studies confirmed the fact that the condition on Guam fulfilled the classical criteria of ALS. However, three major unusual features were noted in these studies: (1) an extraordinarily high incidence, often with familial aggregation1,2; (2) the development, in some instances, of clinical evidence of dementia and extrapyramidal symptomatology3; (3) the presence of histological changes characteristic of the parkinsonismdementia complex (PD), another endemic fatal neurological disease affecting the Chamorros on Guam.4,5 These features were not previously recognized among classical ALS cases. In addition, two clinical features which are not notable among classical ALS cases were discovered as a result of several investigations, including

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