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

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Abstract
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 Recklinghausen9s 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.

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