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PubMed2002

Clinical features and changing patterns of neurodegenerative disorders on Guam, 1997-2000.

BACKGROUND: In the 1950s, high-incidence ALS and Parkinson-dementia complex (PDC) were identified among Chamorros, the native inhabitants of Guam. Brains of patients with these syndromes showed widespread neurofibrillary tangles. Although ALS and PDC were reported to have dramatically declined in the 1980s, new cases are still encountered. Late-life dementia has received little study among Chamorros. METHODS: From 1997 to 2000, the authors evaluated newly referred and previously identified patients. They screened first-degree relatives of previous registries, and subjects aged 60 or older. Subjects who scored below a cognitive test cutoff or had symptoms or signs consistent with parkinsonism or ALS underwent psychometric testing, assessment by a neurologist, and laboratory studies as appropriate. Consensus diagnoses were made. RESULTS: The authors identified 194 Chamorros with ALS (n = 10), PD (n = 11), PDC (n = 90), or late-life dementia (n = 83). Mean ages at onset were 55 for ALS, 68 for PDC, 63 for PD, and 74 for dementia. Late-life dementia was more common in women, and met criteria for probable or possible AD. The APOE-epsilon 4 allele frequency was uniformly low regardless of neurologic diagnosis. CONCLUSIONS: The rapid decline of high-incidence ALS on Guam over the past 40 years suggests the contribution of a modifiable environmental factor. PDC remains relatively common, with an unchanged clinical picture apart from later age at onset. Dementia among elderly Chamorros (termed "Mariana dementia") resembles AD. Autopsy studies will clarify whether this dementia is related to AD pathology or represents a late-life neurofibrillary tangle syndrome more closely allied to PDC.

Age of Onset↗

PubMed2002

Life event timing and the emotional consequences of surgical menopause for Asian-Pacific women in Guam.

Using Behavior Risk Factor Survey data (N = 398), this study tests the age-norms hypothesis by examining the emotional consequences of the timing of surgical menopause for women in Guam's Asian-Pacific community. The results of the study are supportive of the hypothesis that off-scheduled life events result in considerable emotional trauma, and show that women in Guam who are 44 years of age or younger are significantly more likely than older women to suffer psychological distress and unhappiness when they report experiencing surgical menopause. We discuss the potential implications of these findings for theory and future research.

Adult↗

PubMed2004

Asian/Pacific Islander adolescent sexual orientation and suicide risk in Guam.

OBJECTIVES: We examined the effects of same-sex orientation on suicide risks for Guam's Asian/Pacific Islander adolescents. METHODS: We used a probability sample and logistic regression analysis to identify suicide risk factors. RESULTS: Same-sex orientation was associated with a greater risk of suicide attempt, especially for boys. Adolescents who reported suffering physical abuse in the context of a romantic relationship, engaging in binge drinking, and experiencing feelings of hopelessness were at greater risk for suicidal ideation and attempts. Race/ethnicity was associated with suicide risk for both boys and girls, and patterns suggest that membership in the same racial/ethnic group decreased suicide risk for girls and increased risk of suicide for boys. CONCLUSIONS: Gay, lesbian, and bisexual Asian/Pacific Islander adolescents in Guam deserve intervention and counseling programs to reduce suicide risk.

Adolescent↗

PubMed1976

Abnormal carbohydrate metabolism in amyotrophic lateral sclerosis and Parkinsonism-dementia on Guam.

Amyotrophic lateral sclerosis and Parkinsonism-dementia are unusually prevalent on Guam. Carbohydrate metabolism was studied in 110 patients with evidence diagnostic of or suspecious for these diseases. The combined incidence of known diabetes in 29 per cent of them plus a high percentage of glucose tolerance tests interpreted as abnormal, even when most age-related criteria were considered, was considerably higher than the incidence of abnormal carbohydrate metabolism reported elsewhere in the general population of the United States, the tropical Pacific area, or in recent surveys on Guam itself. The diabetes was generally mild in nature and noteworthy for a lack of retinopathy and other complications. Hypertension, hypercholesterolemia, and hyperuricemia, although highly prevalent, were not consistently associated with abnormal glucose metabolism. Similarly, no consistent association was demonstrated with such factors as age, muscle atrophy, or physical activity.

Adult↗

PubMed2000

[An autopsy of parkinsonism after solitary living in Guam Island for 28 years].

The autopsy findings of an 82-year-old man with history of solitary living in the jungle of Guam, the endemic area of parkinsonism-dementia complex(PDC), for 28 years was reported in this paper. When he was 75 years old, about 20 years later to have come back to Japan, he developed parkinsonism. He noticed bradykinesia and was pointed out masked face, rigidity and tremor in his right hand. After 2 years, he was diagnosed as Parkinson's disease under the third degree of Hoehn-Yahr criteria. He also showed mild cognitive dysfunction, but no pyramidal signs, muscle atrophy of fasciculation at all. Anti-parkinsonian drugs were effective for his motor symptoms. He admitted at age 82 because of anorexia, and died after 3 months. Neuropathological study disclosed neuronal loss and gliosis with Lewy bodies in the substantia nigra, locus coeruleus and dorsal vagal nucleus. There were cortical type Lewy bodies in the limbic system and scanty amount in the neocortex. A few neurofibrillary tangles(NFT) were found in the hippocampus and parahippocampal gyrus, but no dominancy in the second or third layers of the cerebral cortex as reported in PDC. Senile plaques were not observed at all. Although the exact cause of PDC has not been clarified, environmental factors such as water or food seem to influence on the outcome of PDC. However, the pathological findings of the present case were compatible to those of idiopathic Parkinson's disease. Thus it is a very important fact that the present case was not suffered from PDC in spite of his long residence in the endemic area of Guam.

Aged↗

PubMed1992

Are the endemic motor neuron diseases of Guam really disappearing?

Death certificates were reviewed to observe trends in the number of persons on Guam reported to have chronic degenerative motor neuron diseases (amyotrophic lateral sclerosis, parkinsonism-dementia or Parkinson's disease) at the time of death. Additional data, including age and race of the deceased and the name of the certifying physician were also collected. The number of persons having been diagnosed with amyotrophic lateral sclerosis is apparently decreasing while their mean age is increasing suggesting that the etiologic agents or factors causing this disease are less prevalent on Guam today than they have been in the past. Trends with regard to parkinsonism-dementia and Parkinson's disease are less clear.

Age Factors↗

PubMed2002

Cholera control on Guam, 2000.

During April, 2000, the island of Pohnpei began experiencing an outbreak of cholera and during June and July of the same year four cases of cholera representing 3 separate introduction events were identified on Guam. Two of these events were associated with eating reef fish imported from Pohnpei. Following the imposition of a narrowly-focused ban on the importation of inshore seafood and processed food products from Pohnpei, no additional local or imported cases of cholera were detected on Guam.

Adult↗

PubMed1986

Observations on the clinical presentations and the neuropathological findings of amyotrophic lateral sclerosis in Australia and Guam.

Among 20 consecutive autopsies of amyotrophic lateral sclerosis (ALS) occurring in Caucasians in Western Australia (WA), 85% were males. The median age of onset was 58.9 years and the average duration of illness was 2.4 years. Twenty-two randomly selected ALS occurring among natives in Guam also showed a male predominance of 75%, younger age of onset (median 48.5 years) and longer survival period (median 3.4 years). 45% of the WA patients presented with bulbar involvement at the time of first examination. These patients had the lowest median survival period of 1.5 years when compared with the other forms of ALS, the classic upper and lower motor system involvement and progressive muscular atrophy. Theneuropathologic lesions of ALS in WA and Guam were similar with the exception that neurofibrillary tangles were frequently present in the Guamanian brains. In 14%, neuronal loss, gliosis and frequency of tangles in the cerebral cortex especially in Ammon's horn, substantia nigra, and locus ceruleus, were sufficiently severe to indicate the coexistence of another disorder, Parkinsonism-Dementia Complex. This condition was not clinically recognized. In the WA cases only one patient had tangles in the brain and he had concurrent Alzheimers disease. While senile plaques were present in this patient they were usually absent in the Guamanian brains.

Adolescent↗

PubMed1980

Salmonella surveillance in Guam.

A high incidence of human Salmonella infections (66/100,000 population for 1978) is reported for the island of Guam in Micronesia. S. waycross, a relatively rare serotype in other areas of the world and first isolated on Guam in 1974, accounted for 39% of all serotyped isolates from human cases in 1978. The possibility of this organism being introduced in imported products is discussed.

Adolescent↗

PubMed1995

Measles outbreak--Guam, 1994.

One of the largest outbreaks of measles in the United States and its territories since 1992 occurred in Guam during 1994. From February 8 through June 25, 1994, a total of 280 suspected, probable, or confirmed cases of measles were reported to the Guam Department of Public Health and Social Services (GDPH). Of these cases, 228 were considered confirmed, including 47 serologically confirmed cases (Figure 1). This report summarizes findings from the investigation of these 228 cases.

Adolescent↗

PubMed1995

Neurofibrillary degeneration in amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam. Immunochemical characterization of tau proteins.

Neurofibrillary tangles are observed in several neurodegenerative disorders including Alzheimer's disease, progressive supranuclear palsy, and amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam. The major components of neurofibrillary tangles are hyperphosphorylated tau proteins that can be directly detected in brain homogenates, using immunoblotting with specific immunological probes. To investigate whether tau proteins differ biochemically among various neurodegenerative disorders, we analyzed a series of brain samples from Guamanian patients in comparison with Alzheimer's disease, progressive supranuclear palsy, and normal aging. In Alzheimer's disease, these hyperphosphorylated tau proteins are composed of a triplet referred to as tau 55, 64, and 69, whereas in progressive supranuclear palsy, neurofibrillary degeneration is characterized by a tau doublet (tau 64 and 69). In the present study, characterization of tau proteins was performed by immunoblotting, on different cortical and subcortical regions of postmortem brain specimens from Guamanian natives. In all of the cases, biochemical data were always consistent with neuropathological findings. In contrast to Alzheimer's disease patients where the tau triplet is found mostly in cortical regions, a similar triplet was strongly detected in both cortical and subcortical areas in Guamanian patients. The tau profile differed quantitatively from case to case demonstrating that the Alzheimer's disease-related tau triplet had a heterogeneous regional distribution. These data suggest that the tau triplet found in amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam is similar to that observed in Alzheimer's disease, and the regional distribution of tau proteins differs in these disorders.

Adult↗

PubMed1981

Population genetic studies of the Philippine Negritos. III. Identification of the carbonic anhydrase-1 variant with CA1 Guam.

Investigation of blood samples from 277 Mamanwas of northeastern Mindanao, Philippines, confirmed the concentration of the variant carbonic anhydrase-1 (CA1 3N) in this group. The frequency for the variant allele was estimated at .217 +/- .017. It occurs also in the Manobos, the Mongoloid indigenous inhabitants of the same district, although the frequency is low (.019 +/- .008). Survey of samples from other Philippine populations, including the Aeta and the Ifugao of Luzon, failed to find variants. This findings suggests different origins of the Aeta and the Mamanwa, although both are usually referred to as Negritos. The Ca1 3N protein was purified by affinity chromatography using azosulfonamide and rechromatography on a DEAE-Sephadex column. The tryptic peptide pattern of CA1 3N was similar to that of CA1 Guam already reported. Furthermore, amino acid analyses of the tryptic peptides indicated that CA1 3N is characterized by the substitution 253 Gly leads to Arg, confirming the identity of this variant with CA1 Guam. The widespread occurrence of CA1 3 variants in the Western Pacific suggests that this variant was once common in an aboriginal population of this region, from which it was scattered by gene flow.

Black People↗

PubMed1990

Geographic patterns of parkinsonism-dementia complex on Guam. 1956 through 1985.

Average annual age-adjusted incidence rates of parkinsonism-dementia complex were obtained for the 19 election districts of Guam from 1956 through 1985. The highest rates were found in the southern and central districts, and the lowest rates were found in the northern and western districts. Geographic and temporal patterns of incidence were associated with socioeconomic status but not with geochemical factors. The risk of parkinsonism-dementia complex in susceptible sibships was much higher than that in the general population--even in districts with the highest incidence rates, but especially in districts with the lowest incidence rates. Our evidence tends to support the hypothesis that multiple factors linked to cycad use play an important role in the cause of PDC. Hypotheses related to metal exposure and simple genetic factors were unsatisfactory explanations for the epidemiologic patterns observed.

Dementia↗

PubMed2004

Genome-wide analysis of the parkinsonism-dementia complex of Guam.

BACKGROUND: Parkinsonism-dementia complex (PDC) is a neurofibrillary tangle degeneration involving the deposition of Alzheimer-type tau, predominantly in the mesial temporal cortex, brainstem, and basal ganglia. It occurs in focal geographic isolates, including Guam and the Kii peninsula of Japan. The familial clustering of the disease has suggested that a genetic factor could be important in its etiology. OBJECTIVE: To determine whether a genetic locus could be identified, linked, or associated with PDC. DESIGN AND PATIENTS: We performed a genome-wide association study of 22 Guamanian PDC and 19 control subjects using 834 microsatellite markers with an approximate genome-wide marker density of 4.4 centimorgans. RESULTS: Two-point association analysis identified 17 markers (P<.015). Each of these markers then underwent conventional linkage analysis in 5 families with PDC. One marker, D20S103, generated a logarithm of odds score of greater than 1.5. Multipoint association analysis also highlighted 2 other areas on chromosome 14q (adjacent to D14S592, 59.2 megabases [M]) and chromosome 20 (adjacent to D20S470, 17.4 M) with multipoint association logarithm of the odds scores of greater than 2. The areas around D20S103, D14S592, and D20S470 were further analyzed by association using additional microsatellite markers and by conventional linkage analysis. This did not provide further evidence for the role of these areas in PDC. CONCLUSIONS: This study has not identified a single gene locus for PDC, confirming the impression of a geographic disease isolate with a complex genetic, a genetic/environmental etiology, or a purely environmental etiology.

Aged↗

PubMed1997

Skeletal biology of Apurguan: a precontact Chamorro site on Guam.

The human skeletal remains of a minimum of 152 individuals from the precontact Latte Period (AD 1000-1521) on Guam, Mariana Islands, are described. The sample, recovered at Apurguan, in the Tamuning District, is one of the largest series of well-provenienced Chamorro skeletal remains to be analyzed in recent years. The size and systematic nature of this database are a major contribution to the human biology of the region. Paleodemographic characteristics, dental and skeletal morphology, and paleopathology are presented, along with a limited examination of sex differences in frequencies of nonmetric variation. The mortuary sample, consisting of 51 subadults and 101 adults, exhibits underrepresentation of females, highest subadult mortality between 2 and 10 years, and an adult average age-at-death of 43.5 years. Cranial and infracranial indices and nonmetric variation are consistent with the Chamorro's Southeast Asian origins. There are few statistically significant sex differences in nonmetric variation which suggests close genetic affinity. The frequency of dental pathology overall is low, reflecting a well-balanced, varied diet, and consistent with preagricultural subsistence; however statistically significant sex differences suggest the influence of differential cultural behaviors or resource access. Paleopathological observations include healed fractures (more common in males), little advanced osteoarthritis, evidence for gouty arthritis, and treponemal disease (yaws). One individual, a young adult male, was interred with 10 human bone spear points in situ. Twenty percent of the primary burials exhibit evidence of postdepositional removal of selected skeletal elements for cultural purposes such as keepsakes or raw material.

Arthritis↗

PubMed1997

Subadult stress, morbidity, and longevity in Latte Period populations on Guam, Mariana Islands.

The frequency and age distribution of linear enamel hypoplasia (LEH) in the dentition of 293 individuals from Latte Period sites (AD 800-1521) on Guam, Mariana Islands, are examined in this study. Individuals dying as subadults (before age 16) and as young adults (ages 16-21) have more frequent LEHs than those who survived to middle or late adulthood, documenting a relationship between LEH-causing stress events and reduced life expectancy. The age distributions of cribra orbitalia and skeletal infection in children who died by age 10 exhibit striking similarities to the etiological age patterns of LEH in children, and those with skeletal infection have more frequent hypoplasias than children without infection. The comorbidity of systemic stress and infection in children, and their impact on life expectancy, are interpreted in the biocultural context of high population density in the large coastal villages of the late prehistoric period in the Marianas.

Adult↗

PubMed1997

Spondylolysis in prehistoric human remains from Guam and its possible etiology.

This study reports the findings of complete bilateral separation of the neural arch (spondylolysis) in 176 inhumations from the Hyatt Site, Tumon Bay, which is located on the west side of the island of Guam. Skeletons were excavated and analyzed by the Paul H. Rosendahl Inc. (PHRI) team in 1989-1990. The inhumations were associated with the pre-European Latte Period (circa 1,200-1,521 A.D.). This period was characterized by the use of large stone pillars, called latte sets, for the construction of houses. Of the 176 individuals, only 38 adult skeletons had complete spines, and 21% (8/38) of these had evidence of spondylolysis in their lumbar vertebrae, particularly in L-5. The age of the eight individuals range from 30 to 50 years. No children were found with spondylolysis. Of the males 29.4% (5/17) had spondylolysis, as did 14.3% (3/21) of the females. However, the difference between the sexes was not statistically significant. Though the sample is small, it is suggested that the high incidence of lumbar spondylolysis found in these ancient Chamorros was related to lower back traumatic events. The transport of latte stones, involving hyperextension and torque of the lower back, while dragging the stones, probably contributed to the development of microfractures in the spine and subsequent spondylolysis. If this hypothesis is correct, then both males and females appear to have been participants in an organized community labor force. It is predicted that similar frequencies of spondylolysis will be found at other Latte sites.

Bone and Bones↗

PubMed1983

Search for a red cell enzyme or serum protein marker in amyotrophic lateral sclerosis and parkinsonism-dementia of Guam.

Frequency distributions were determined for 24 red cell enzyme and four serum protein systems, in an attempt to identify a genetic marker associated with either amyotrophic lateral sclerosis (ALS) or parkinsonism-dementia (PD), two progressive and fatal neurological disorders of unknown cause found with unusually high incidence among the Chamorros of Guam and the Northern Mariana Islands. No striking associations were identified between either disorder and any of the gene markers tested. Thus, no genetic cause is known for either disease; local environmental factors are most likely involved in pathogenesis.

Amyotrophic Lateral Sclerosis↗