Amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam (Q86928): Difference between revisions
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Uma alta taca de prevalência de ELA e complexo de Parkinson-demência (PDC) foi observado no povo Chamorro de Guam. Os pacientes que apresentam doença com predominância motora apresentam características consistentes com ELA clássica, e uma proporção menor desses pacientes pode apresentar demência sobreposta ou parkinsonismo. Pacientes com PDC comumente demonstram manifestações de disfunção do sistema motor, como fraqueza dos membros, fasciculações e hiperreflexia. Existem agrupamentos familiares de doenças, mas os padrões de herança genética não foram definidos. Neuropatologia única foi identificada com alterações cerebrais típicas de ALS misturadas com acúmulos de emaranhados neurofibrilares. A incidência de ELA e PDC caiu recentemente. | |||
| description / en | description / en | ||
A high prevalence focus of ALS and Parkinsons-dementia complex (PDC) has been noted in the Chamorro people of Guam. Patients presenting with motor-predominant disease display features consistent with classical ALS, and a smaller proportion of these patients may display superimposed dementia or parkinsonism. Patients with PDC commonly demonstrate manifestations of motor system dysfunction such as limb weakness, fasciculations and hyperreflexia. There are familial groupings of disease but genetic inheritance patterns have not been defined. Unique neuropathology has been identified with brain changes typical of ALS admixed with accumulations of neurofibrillary tangles. The incidence of ALS and PDC has fallen recently. | |||
Revision as of 12:30, 15 August 2026
A high prevalence focus of ALS and Parkinsons-dementia complex (PDC) has been noted in the Chamorro people of Guam. Patients presenting with motor-predominant disease display features consistent with classical ALS, and a smaller proportion of these patients may display superimposed dementia or parkinsonism. Patients with PDC commonly demonstrate manifestations of motor system dysfunction such as limb weakness, fasciculations and hyperreflexia. There are familial groupings of disease but genetic inheritance patterns have not been defined. Unique neuropathology has been identified with brain changes typical of ALS admixed with accumulations of neurofibrillary tangles. The incidence of ALS and PDC has fallen recently.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_1780993349 |
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| English | Amyotrophic lateral sclerosis-parkinsonism-dementia complex of Guam |
A high prevalence focus of ALS and Parkinsons-dementia complex (PDC) has been noted in the Chamorro people of Guam. Patients presenting with motor-predominant disease display features consistent with classical ALS, and a smaller proportion of these patients may display superimposed dementia or parkinsonism. Patients with PDC commonly demonstrate manifestations of motor system dysfunction such as limb weakness, fasciculations and hyperreflexia. There are familial groupings of disease but genetic inheritance patterns have not been defined. Unique neuropathology has been identified with brain changes typical of ALS admixed with accumulations of neurofibrillary tangles. The incidence of ALS and PDC has fallen recently. |
