Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam.
Identifieur interne : 001820 ( PubMed/Curation ); précédent : 001819; suivant : 001821Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam.
Auteurs : P L Mcgeer [Canada] ; C. Schwab ; E G Mcgeer ; R L Haddock ; J C SteeleSource :
- Neurology [ 0028-3878 ] ; 1997.
English descriptors
- KwdEn :
- Aged, Aged, 80 and over, Amyotrophic Lateral Sclerosis (genetics), Amyotrophic Lateral Sclerosis (mortality), Amyotrophic Lateral Sclerosis (pathology), Brain (pathology), Dementia (genetics), Dementia (mortality), Dementia (pathology), Female, Guam (ethnology), Humans, Male, Middle Aged, Mortality, Parkinson Disease (genetics), Parkinson Disease (mortality), Parkinson Disease (psychology), Syndrome.
- MESH :
- geographic , ethnology : Guam.
- genetics : Amyotrophic Lateral Sclerosis, Dementia, Parkinson Disease.
- mortality : Amyotrophic Lateral Sclerosis, Dementia, Parkinson Disease.
- pathology : Amyotrophic Lateral Sclerosis, Brain, Dementia.
- psychology : Parkinson Disease.
- Aged, Aged, 80 and over, Female, Humans, Male, Middle Aged, Mortality, Syndrome.
Abstract
Chamorros suffer from two neurologic syndromes known as ALS and the parkinsonism-dementia complex (PDC) of Guam. We report mortality figures for these syndromes during 1991 to 1995 and compare them with those at 5-year intervals dating back to 1951. In contrast to predictions of disease disappearance, both syndromes remain prevalent. However, age of onset and age at death have increased for both syndromes, suggesting that shifting environmental factors are causing disease postponement. We also report the clinical, familial, neuropathologic, and immunohistochemical findings on a consecutive autopsy series of Guamanian Chamorro cases. Twelve cases were diagnosed as PDC, known locally as "bodig," and three as ALS, known locally as "lytico." All but three of these fifteen patients had a pronounced family history of similar illness. Eight of twelve boding patients had siblings who were also affected with bodig; two of three lytico cases had siblings afflicted with lytico. The family histories are compatible with genetic transmission of each syndrome. The neuropathology of bodig is characterized by severe and widespread neurofibrillary tangle (NFT) development. NFTs are surrounded by reactive microglia and reactive astrocytes, and complement proteins and other molecules connected with inflammation are associated with them. Similar inflammatory responses also occur in Alzheimer's disease (AD) but have been largely attributed to the presence of senile plaques. These data indicate that tangles, as well as plaques, generate inflammatory reactions that such reactions may exacerbate the fundamental pathology in bodig as well as in AD.
PubMed: 9270568
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Schwab</name></author><author><name sortKey="Mcgeer, E G" sort="Mcgeer, E G" uniqKey="Mcgeer E" first="E G" last="Mcgeer">E G Mcgeer</name></author><author><name sortKey="Haddock, R L" sort="Haddock, R L" uniqKey="Haddock R" first="R L" last="Haddock">R L Haddock</name></author><author><name sortKey="Steele, J C" sort="Steele, J C" uniqKey="Steele J" first="J C" last="Steele">J C Steele</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1997">1997</date><idno type="RBID">pubmed:9270568</idno><idno type="pmid">9270568</idno><idno type="wicri:Area/PubMed/Corpus">001820</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001820</idno><idno type="wicri:Area/PubMed/Curation">001820</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">001820</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam.</title><author><name sortKey="Mcgeer, P L" sort="Mcgeer, P L" uniqKey="Mcgeer P" first="P L" last="Mcgeer">P L Mcgeer</name><affiliation wicri:level="1"><nlm:affiliation>Kinsmen Laboratory of Neurological Research, University of British Columbia, Vancouver, Canada.</nlm:affiliation><country xml:lang="fr">Canada</country><wicri:regionArea>Kinsmen Laboratory of Neurological Research, University of British Columbia, Vancouver</wicri:regionArea></affiliation></author><author><name sortKey="Schwab, C" sort="Schwab, C" uniqKey="Schwab C" first="C" last="Schwab">C. Schwab</name></author><author><name sortKey="Mcgeer, E G" sort="Mcgeer, E G" uniqKey="Mcgeer E" first="E G" last="Mcgeer">E G Mcgeer</name></author><author><name sortKey="Haddock, R L" sort="Haddock, R L" uniqKey="Haddock R" first="R L" last="Haddock">R L Haddock</name></author><author><name sortKey="Steele, J C" sort="Steele, J C" uniqKey="Steele J" first="J C" last="Steele">J C Steele</name></author></analytic><series><title level="j">Neurology</title><idno type="ISSN">0028-3878</idno><imprint><date when="1997" type="published">1997</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Aged</term><term>Aged, 80 and over</term><term>Amyotrophic Lateral Sclerosis (genetics)</term><term>Amyotrophic Lateral Sclerosis (mortality)</term><term>Amyotrophic Lateral Sclerosis (pathology)</term><term>Brain (pathology)</term><term>Dementia (genetics)</term><term>Dementia (mortality)</term><term>Dementia (pathology)</term><term>Female</term><term>Guam (ethnology)</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Mortality</term><term>Parkinson Disease (genetics)</term><term>Parkinson Disease (mortality)</term><term>Parkinson Disease (psychology)</term><term>Syndrome</term></keywords><keywords scheme="MESH" type="geographic" qualifier="ethnology" xml:lang="en"><term>Guam</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Amyotrophic Lateral Sclerosis</term><term>Dementia</term><term>Parkinson Disease</term></keywords><keywords scheme="MESH" qualifier="mortality" xml:lang="en"><term>Amyotrophic Lateral Sclerosis</term><term>Dementia</term><term>Parkinson Disease</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Amyotrophic Lateral Sclerosis</term><term>Brain</term><term>Dementia</term></keywords><keywords scheme="MESH" qualifier="psychology" xml:lang="en"><term>Parkinson Disease</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Aged</term><term>Aged, 80 and over</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Mortality</term><term>Syndrome</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Chamorros suffer from two neurologic syndromes known as ALS and the parkinsonism-dementia complex (PDC) of Guam. We report mortality figures for these syndromes during 1991 to 1995 and compare them with those at 5-year intervals dating back to 1951. In contrast to predictions of disease disappearance, both syndromes remain prevalent. However, age of onset and age at death have increased for both syndromes, suggesting that shifting environmental factors are causing disease postponement. We also report the clinical, familial, neuropathologic, and immunohistochemical findings on a consecutive autopsy series of Guamanian Chamorro cases. Twelve cases were diagnosed as PDC, known locally as "bodig," and three as ALS, known locally as "lytico." All but three of these fifteen patients had a pronounced family history of similar illness. Eight of twelve boding patients had siblings who were also affected with bodig; two of three lytico cases had siblings afflicted with lytico. The family histories are compatible with genetic transmission of each syndrome. The neuropathology of bodig is characterized by severe and widespread neurofibrillary tangle (NFT) development. NFTs are surrounded by reactive microglia and reactive astrocytes, and complement proteins and other molecules connected with inflammation are associated with them. Similar inflammatory responses also occur in Alzheimer's disease (AD) but have been largely attributed to the presence of senile plaques. These data indicate that tangles, as well as plaques, generate inflammatory reactions that such reactions may exacerbate the fundamental pathology in bodig as well as in AD.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">9270568</PMID><DateCreated><Year>1997</Year><Month>09</Month><Day>17</Day></DateCreated><DateCompleted><Year>1997</Year><Month>09</Month><Day>17</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0028-3878</ISSN><JournalIssue CitedMedium="Print"><Volume>49</Volume><Issue>2</Issue><PubDate><Year>1997</Year><Month>Aug</Month></PubDate></JournalIssue><Title>Neurology</Title><ISOAbbreviation>Neurology</ISOAbbreviation></Journal><ArticleTitle>Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam.</ArticleTitle><Pagination><MedlinePgn>400-9</MedlinePgn></Pagination><Abstract><AbstractText>Chamorros suffer from two neurologic syndromes known as ALS and the parkinsonism-dementia complex (PDC) of Guam. We report mortality figures for these syndromes during 1991 to 1995 and compare them with those at 5-year intervals dating back to 1951. In contrast to predictions of disease disappearance, both syndromes remain prevalent. However, age of onset and age at death have increased for both syndromes, suggesting that shifting environmental factors are causing disease postponement. We also report the clinical, familial, neuropathologic, and immunohistochemical findings on a consecutive autopsy series of Guamanian Chamorro cases. Twelve cases were diagnosed as PDC, known locally as "bodig," and three as ALS, known locally as "lytico." All but three of these fifteen patients had a pronounced family history of similar illness. Eight of twelve boding patients had siblings who were also affected with bodig; two of three lytico cases had siblings afflicted with lytico. The family histories are compatible with genetic transmission of each syndrome. The neuropathology of bodig is characterized by severe and widespread neurofibrillary tangle (NFT) development. NFTs are surrounded by reactive microglia and reactive astrocytes, and complement proteins and other molecules connected with inflammation are associated with them. Similar inflammatory responses also occur in Alzheimer's disease (AD) but have been largely attributed to the presence of senile plaques. 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