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Amyotrophic lateral sclerosis and diabetes on Guam: changing patterns of chronic disease in an island community.

Amyotrophic lateral sclerosis (ALS) occurred on the island of Guam with unusually high incidence rates for many years but began to disappear with the island's westernization after WW II. The authors document these changes and suggest that they support a hypothesis that cultural changes could be responsible for both the virtual disappearance of this chronic degenerative neurologic disease as well as a concurrent surge in the prevalence of diabetes.

Amyotrophic Lateral Sclerosis↗

A search for infant salmonellosis risk factors on Guam.

A study was conducted in the hope of identifying risk factors associated with a 10-year epidemic of salmonellosis among infants on Guam. Salmonella bacteria were found in soil and vacuum cleaner samples suggesting that Salmonella infections may result from contamination introduced into homes from the outdoor environment by mechanical means rather than by the ingestion of contaminated animal-origin foods.

Environmental Microbiology↗

NAG Vibrio cholerae isolated from imported shellfish in Guam.

A survey of imported shellfish available in public markets on the Island of Guam revealed the presence of NAG Vibrio cholerae contamination (10 of 38 sample lots positive) and high coliform counts (21 of 33 sample lots in excess of 2400 per 100 grams). NAG V. cholerae contamination was associated with high coliform counts and origin in fresh or brackish waters rather than saltwater. Further importation of fresh shellfish from other than approved sources was prohibited.

Food Contamination↗

Neuropathology of the cortical lesions of the Parkinsonian-dementia (PD) complex of Guam.

The Guam amyotrophic lateral sclerosis and Parkinsonian-dementia (ALS and PD) complex still presents a challenge to research. Previous papers have highlighted the lesions in the hippocampus and basal nuclei to explain the bizarre clinical syndrome. In our study of 20 to 62 cases of PD with or without ALS, significant widespread and often severe lesions were found throughout the entire cerebral cortex. These changes included variable degrees of nerve cell loss, spongy change, gliosis, increased lipofuscin content of nerve cells, the presence of granulovacuolar bodies and neurofibrillary tangles within neurones and, less frequently, Hirano bodies. The lesions show a characteristic topographical distribution. The global involvement of the cerebral cortex correlated well with the loss of higher mental function which was present in the patients.

Adult↗

The origin of non-outbreak Vibrio parahaemolyticus infections on Guam.

A study of Vibrio parahaemolyticus infected patients not associated with known outbreaks and controls matched for sex, ethnicity and age (+/- 5 years) was conducted on Guam. Cases were asked if they had eaten seafood within the 24-hour period preceding onset of illness and controls were asked if they had eaten seafood within the 24-hour period preceding their interview. Cases were more likely than controls to have eaten seafood (OR = 37.59, CL [8.30-220.24]).

Case-Control Studies↗

Spauligodon gehyrae n. sp. (Nematoda:Pharyngodonidae) from Gehyra oceanica (Sauria:Gekkonidae) from Guam, Mariana Islands, Micronesia.

Spauligodon gehyrae n. sp. (Nematoda: Pharyngodonidae), a new oxyurid nematode, discovered in the large intestine of the gecko Gehyra oceanica is described and illustrated. Nine of 11 adult female specimens of G. oceanica collected on Guam, Mariana Islands, Micronesia, harbored a total of 480 specimens of S. gehyrae; prevalence of infection was 82% (mean intensity 53, range 6-179). Spauligodon gehyrae represents the 33rd species to be described for this genus and is distinguished by the presence of truncated egg ends, the short stiff tail spike of the female, and the presence of a spicule in the male. This is the second species of Spauligodon to be described from Oceania.

Animals↗

Troubles in Smurftown: youth gangs and moral visions on Guam.

An explosive youth gang problem in U.S. Guam may derive from the changes in island traditionalism wrought by modern society. Island youths sense the erosion of the traditional culture's moral visions, but find modern Western individualism unpalatable as an alternative. Gang membership offers a third option, bestowing on youths a sense of honor, bravery, and solidarity lost from traditional society. Gang members must be viewed not as social deviants but as members of a culture that proposes moral visions and prescribes group behaviors for its membership.

Child↗

Polymarker, HLA-DQA1, and D1S80 allele frequency data in Chamorro and Filipino populations from Guam.

Allele frequencies were determined in sample populations of Chamorros and Filipinos from Guam at the loci LDLR, GYPA, HBGG, D7S8, Gc, HLA-DQA1, and D1S80. Variable number tandem repeat alleles at the D1S80 locus were detected by silver staining following electrophoresis of amplified products in polyacrylamide. Allelic products of the other loci were detected by reverse dot blot hybridization following a multiplex amplification procedure. All loci, in both sample populations, are highly polymorphic and meet Hardy-Weinberg expectations, except for the D1S80 locus in the Chamorro population sample (p = 0.025). An interclass correlation analysis detected only one marginally significant departure from independence out of a total of 42 pairwise comparisons of the seven loci for both data sets (LDLR/HBGG in Chamorros, p = 0.048). The Chamorro and Filipino allele frequency data are similar to each other at six of the seven loci with only a marginally significant difference at the HLA-DQA1 locus (p = 0.049).

Alleles↗

Midkine-like immunoreactivity in extracellular neurofibrillary tangles in brains of patients with parkinsonism-dementia complex of Guam.

Midkine (MK) has been shown to be present in amyloid deposits in Alzheimer's disease (AD). In the present study, expression of midkine was examined immunohistochemically in brains of patients with the parkinsonism-dementia complex of Guam, lytico bodig disease (LB), using an affinity purified antibody to midkine. Positive staining was identified on some extracellular neurofibrillary tangles. The relative prevalence was somewhat less than for beta-amyloid protein (Abeta)-positive extracellular tangles. The results demonstrate that expression of MK and Abeta is not an exclusive feature of amyloid deposits in Alzheimer brain, but is also found in LB brain.

Antibody Specificity↗

On the survival time of a tangled neuron in the hippocampal CA4 region in parkinsonism dementia complex of Guam.

In diseases such as the Parkinson dementia complex of Guam (PDC) or Alzheimer's disease, susceptible neurons develop intracellular tangles (iNFTs) and then die, leaving behind extracellular tangles (eNFTs). We performed counts of healthy neurons, iNFTs, and eNFTs in the hippocampus of Guamanian Chamorros who were neurologically normal or who suffered from PDC. The total of surviving and dead neurons in the CA4 region was remarkably constant from case to case, indicating that eNFTs are not phagocytosed. Since cases of recent PDC showed only marginal tangle formation in CA4, we concluded that tangle development in CA4 commenced close to the onset of the disease. Based on this assumption, as well as the further assumption that the average rate of tangle development and the average lifetime of a tangled neuron do not alter as the disease progresses, we derived equations to determine the average lifetime of tangled neurons. The results varied from 0.13 years for the most rapidly progressing case to 7.98 years for the most slowly developing case. The average for 8 cases was 2.51 years.

Adult↗

Genetic variation at nine short tandem repeat loci in Chamorros and Filipinos from Guam.

Allele distributions for nine tetrameric short tandem repeat (STR) loci D3S1358, D5S818, D7S820, D8S1179, D13S317, D18S51, D21S11, FGA and vWA were determined in Chamorros and Filipinos residing in Guam. There was little evidence for departures from Hardy-Weinberg expectations (HWE) in any of the populations. Based on the exact test, the only locus that departs significantly from HWE is D8S1179 (p=0.005, Chamorros; p=0.030, Filipinos). There is little evidence for association of alleles between the loci in these databases. The number of pairwise locus departures from expectations is no more than would be expected by chance. The allelic frequency data are similar to comparable published data within the same population group (e.g., a comparison of Filipinos with Filipinos) and significantly different compared with other major population groups, such as African Americans, Caucasians, and Hispanics. The F(ST) estimate over all nine STR loci is 0.0090.

Journal Article↗

New ketodienes from the integumental lipids of the Guam brown tree snake, Boiga irregularis.

A mixture of six new long chain ketodienes, (6Z,26Z)-pentatriacontadien-2-one [1], (8Z,26Z)-pentatriacontadien-2-one [2], (6Z,27Z)-hexatriacontadien-2-one [3], (8Z,27Z)-hexatriacontadien-2-one [4], (6Z,28Z)-heptatriacontadien-2-one [5], and (8Z,28Z)-heptatriacontadien-2-one [6], has been separated from the cuticular lipids of the Guam brown tree snake Boiga irregularis. Their structures were determined by chemical and spectral means.

Alkadienes↗

Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam, 1945-1972. I. Descriptive epidemiology.

An overview of the epidemiologic studies of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) from 1945 through 1972 is presented. During this period 350 cases of ALS were documented. PD, which is apparently unique to the native Chamorro population, was not recognized during the early years of the study. A total of 213 PD patients have now been seen. The rates of both diseases have declined by approximately 50 per cent since 1965. In the early years incidence per 100,000 for ALS males approached 60; for females it was about 40. For PD males it was about 50; for females it was close to 20. The declines in both diseases have occurred in both sexes and at all age groups and no cohort phenomenon was observed. Marked geographic differences in the distribution of the diseases were observed with southern villages having the highest rates and western villages having the lowest rates; the remainder of the island was intermediate. Recent declines have been most marked in the southern high-rate villages. Chamorros living on the island of Rota have rates similar to those on Guam; those on Saipan have lower rates. A possible excess of ALS among Filipino residents ofGuam was noted; ALS has not been seen among other ethnic groups. Geographic mapping even in high incidence areas did not reveal true clusters or foci. Extensive case-control studies did not reveal any patterns of prior illness, life-style, or exposures distinguishing patients. A tendency for patients to be of somewhat lower socioeconomic level, have less education, eat more homegrown foods and raw meats, and more contact with animals was found. No Mendelian genetic patterns were observed; males were affected more frequently than female for both diseases. Cases did not occur before age 20, reached maximum frequency between ages 55 and 65 and there-after declined. Environmental factors associated with some aspects of the traditional way of life seem to be causally involved, but, since most aspects of the traditional life have changed in the past 50 years, the specific factors remain elusive.

Adult↗

Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam, 1945-1972. II. Familial and genetic studies.

The extraordinarily high rate of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD) in the Chamorro linguistic group on Guam suggested a genetic etiology for these diseases. Results of three studies are described. Two involved comparisons of the rates of disease in relatives and spouses of index patients and controls. In the third we examined offspring of doubly affected parents, 10 spouse pairs with ALS, 3 spouse pairs with PD and 13 spouse pairs in which 1 spouse had ALS and the other PD. Within the limitations of numbers and age of offspring, we found a tendency among siblings and offspring of ALS cases to have a higher risk than control relatives. Numbers were small and in no instance were significant differences encountered. Spouses of patients had at least as high risk of developing ALS or PD as any of the groups of blood relatives. A slightly higher age-specific rate of ALS and PD occurred in the offspring of doubly affected parents. The only group large enough for present analysis are 60 offspring of doubly affected ALS parents over the age of 20. Thus far only 3 have developed ALS. Within our entire patient population of 350 ALS cases and 219 PD cases there were 12 instances in which one parent of the patient was not Chamorro. In addition there is a consistent two- to threefold excess of affected males which could not be accounted for by competitive risks of death from other causes among females. While a familial tendency does exist these findings and observations are not compatible with any simple Mendelian form of inheritance. It is possible that genetic influences affect the occurrence of these diseases but our studies suggest that environmental factors play at least as important a role.

Adult↗

Predicting the duration of Guam amyotrophic lateral sclerosis.

During the years of study of amyotrophic lateral sclerosis on Guam we have observed a wide range in clinical signs and rate of progression of the disease. Some patients died within 6 months of onset, while others have lived for 20 years. It was our assumption that some aspects of the early neurologic involvement would be related to length of survival, and hence be of prognostic value. We found that an early age at onset and male sex were associated with longer survival. The detailed analysis of degree of involvement of four major neurologic components of amyotrophic lateral sclerosis (progressive muscular atrophy, lateral sclerosis, bulbar paralysis, and pseudobulbar palsy) showed no meaningful pattern of association with duration of illness that could be useful in predicting the course.

Adult↗

Relative population densities of Aedes albopictus and A. guamensis on Guam.

Aedes guamensis Farner & Bohart is indigenous to Guam but A. albopictus is known to have arrived in 1944. The larval ecology of the two species is very similar; both species breed in tree holes, coconut shells, and other natural and man-made water containers. The purpose of the study was to compare the relative densities of larval populations of the two species with those determined in a survey made in 1948-49. The results suggest that as the population density of A. albopictus increased, that of A. guamensis decreased by as much as about 95% in artificial containers, and by 30% or more in natural breeding habitats. Since there seems to be no environmental factor involved in rendering the breeding places unsuitable for A. guamensis, the reduction in A. guamensis populations may be, in part, the result of competition from A. albopictus.

Aedes↗