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Clinical and genetic patterns of neurological diseases other than amyotrophic lateral sclerosis on Guam

From the Mayo Graduate School of Medicine and Mayo Clinic, Rochester, Minn., the National Institute of Neurological Diseases and Blindness, Bethesda, Md., and the Mount Sinai School of Medicine, New York City

SUMMARYA study of familial aggregations of neurological diseases was conducted on Guam to determine if these disorders occurred at an unusually high rate and to investigate if these syndromes were related to ALS and PD, which are so frequent in this population. We encountered 39 patients in 4 presumably unrelated families with myotonic dystrophy, 15 patients with Charcot-Marie-Tooth disease in 2 unrelated families, 2 unrelated families with different forms of spinocerebellar degenerations, 1 family with von Recklinghausen's disease, and 1 family with tuberous sclerosis. There was also one sibship of 14 in another family with stigmata of phakomatosis; 4 of the children died of different brain tumors and 2 others died of acute myelogenous leukemia. There is no convincing evidence from the population studies on Guam that the totality of genetic diseases is unusually common or that the neurogenetic diseases which were found have any obvious relationship with ALS and PD.

Reprint requests to Section of Publications, Mayo Clinic, Rochester, Minnesota 55901.

Submitted for publication Jan. 28, 1970; accepted Feb. 16, 1970.

The authors thank the staff of the National Institute of Neurological Diseases and Stroke Research Center of Guam, particularly Francis Leon Guerrero, for collecting family data, and the Department of Vital Statistics, Government of Guam, and Commissioners of Guam. Dr. William Lashey, US. Navy, contributed to the study of myotonic dystrophy. Dr. Hymie Gordon gave valuable criticism on this manuscript.

This article has been cited by other articles:

				L. Olivares, E. S. Esteban, and M. Alter Mexican "Resistance" to Amyotrophic Lateral Sclerosis Arch Neurol, November 1, 1972; 27(5): 397 - 402. [Abstract] [PDF]