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  1. Article ; Online: Amyotrophic lateral sclerosis: update for family physicians.

    Shoesmith, Christen L / Strong, Michael J

    Canadian family physician Medecin de famille canadien

    2006  Volume 52, Issue 12, Page(s) 1563–1569

    Abstract: ... treatment of amyotrophic lateral sclerosis (ALS), a degenerative disorder of the nervous system associated ... searched using the MeSH headings "amyotrophic lateral sclerosis," "therapy," "epidemiology," and "etiology ... There were some level I drug trials.: Main message: Amyotrophic lateral sclerosis is associated ...

    Abstract Objective: To discuss the epidemiology, pathogenesis, diagnosis, expected course, prognosis, and treatment of amyotrophic lateral sclerosis (ALS), a degenerative disorder of the nervous system associated with progressive weakness.
    Quality of evidence: PubMed and the Cochrane Database of Systematic Reviews were searched using the MeSH headings "amyotrophic lateral sclerosis," "therapy," "epidemiology," and "etiology." Articles containing the best available evidence were reviewed. Most provided level II and III evidence. There were some level I drug trials.
    Main message: Amyotrophic lateral sclerosis is associated with progressive dysarthria, dysphagia, and weakness in the extremities. Diagnosis is based on physical examination, electrophysiology, and excluding other confounding conditions. There is no cure for this devastating disorder. Certain treatments, however, can improve survival and quality of life.
    Conclusion: Because ALS is a complex disease, care of ALS patients is best provided at multidisciplinary clinics that specialize in managing patients with this disorder.
    MeSH term(s) Amyotrophic Lateral Sclerosis/diagnosis ; Amyotrophic Lateral Sclerosis/therapy ; Contraindications ; Diagnosis, Differential ; Disease Progression ; Enteral Nutrition ; Family Practice ; Humans ; Nutrition Assessment ; Prognosis
    Language English
    Publishing date 2006-12
    Publishing country Canada
    Document type Journal Article ; Review
    ZDB-ID 2146676-2
    ISSN 1715-5258 ; 0008-350X
    ISSN (online) 1715-5258
    ISSN 0008-350X
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Manifestaciones neuropsiquiátricas y cognitivas en demencia frontotemporal y esclerosis lateral amiotrófica: dos polos de una entidad común.

    Lillo, Patricia / Matamala, José Manuel / Valenzuela, Daniel / Verdugo, Renato / Castillo, José Luis / Ibáñez, Agustín / Slachevsky, Andrea

    Revista medica de Chile

    2014  Volume 142, Issue 7, Page(s) 867–879

    Abstract: ... and amyotrophic lateral sclerosis (ALS) are overlapping multisystem disorders. While 10-15% of ALS ... an update on the recent genetic and neuropathologic findings of ALS and FTD and a characterization ... chromosome 9p21) has been identified in families affected by ALS, ALS-FTD and FTD. This review provides ...

    Title translation Overlapping features of frontotemporal dementia and amyotrophic lateral sclerosis.
    Abstract Recent genetic and neuropathologic advances support the concept that frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are overlapping multisystem disorders. While 10-15% of ALS patients fulfil criteria for FTD, features of motor neuron disease appear in approximately 15% of FTD patients, during the evolution of the disease. This overlap has been reinforced by the discovery of Transactive Response DNA Binding Protein 43 kDa (TDP43) inclusions as the main neuropathologic finding in the majority of ALS cases and almost a half of FTD cases. Also, an expansion in the intron of C9ORF72 (chromosome 9p21) has been identified in families affected by ALS, ALS-FTD and FTD. This review provides an update on the recent genetic and neuropathologic findings of ALS and FTD and a characterization of their clinical presentation forms, based on the current diagnostic criteria. Finally it underscores the importance of having a national registry of patients with ALS and FTD, to provide an earlier diagnosis and a multidisciplinary care.
    MeSH term(s) Amyotrophic Lateral Sclerosis/diagnosis ; Amyotrophic Lateral Sclerosis/genetics ; Amyotrophic Lateral Sclerosis/pathology ; Amyotrophic Lateral Sclerosis/psychology ; DNA Repeat Expansion ; DNA-Binding Proteins/genetics ; Frontotemporal Dementia/diagnosis ; Frontotemporal Dementia/genetics ; Frontotemporal Dementia/pathology ; Frontotemporal Dementia/psychology ; Genotype ; Humans ; Mutation
    Chemical Substances DNA-Binding Proteins
    Language Spanish
    Publishing date 2014-07
    Publishing country Chile
    Document type English Abstract ; Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 732136-3
    ISSN 0717-6163 ; 0034-9887
    ISSN (online) 0717-6163
    ISSN 0034-9887
    DOI 10.4067/S0034-98872014000700007
    Database MEDical Literature Analysis and Retrieval System OnLINE

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