ParkinsonCanadaV1, Main, Exploration, bibRecord, 003C58

Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam

Identifieur interne : 003C58 ( Main/Exploration ); précédent : 003C57; suivant : 003C59

Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam

Auteurs : P. L. Mcgeer [Canada] ; C. Schwab [Canada] ; E. G. Mcgeer [Canada] ; R. L. Haddock ; J. C. Steele

Source :

Neurology [ 0028-3878 ] ; 1997.

RBID : Pascal:97-0494389

Descripteurs français

Pascal (Inist)
- Parkinson démence ile de Guam, Sclérose latérale amyotrophique, Immunohistochimie, Autopsie, Maladie héréditaire, Diagnostic, Etude familiale, Homme, Maladie familiale, Guam.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Aged, Aged, 80 and over, Amyotrophic Lateral Sclerosis (genetics), Amyotrophic Lateral Sclerosis (mortality), Amyotrophic Lateral Sclerosis (pathology), Amyotrophic lateral sclerosis, Autopsy, Brain (pathology), Dementia (genetics), Dementia (mortality), Dementia (pathology), Diagnosis, Family study, Female, Genetic disease, Guam (ethnology), Guam Parkinson dementia, Human, Humans, Immunohistochemistry, 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

Article 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 bodig 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 and that such reactions may exacerbate the fundamental pathology in bodig as well as in AD.

Affiliations:

Canada

Le document en format XML

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<front><div type="abstract" xml:lang="en">Article 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 bodig 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 and that such reactions may exacerbate the fundamental pathology in bodig as well as in AD.</div>
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La maladie de Parkinson au Canada (serveur d'exploration)

Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam

Familial nature and continuing morbidity of the amyotrophic lateral sclerosis-parkinsonism dementia complex of Guam

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri

	La maladie de Parkinson au Canada (serveur d'exploration)
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