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  1. Article ; Online: Creutzfeldt-Jakob disease: a rare cause of dementia in elderly persons.

    Knight, Richard

    Clinical infectious diseases : an official publication of the Infectious Diseases Society of America

    2006  Volume 43, Issue 3, Page(s) 340–346

    Abstract: ... persons, all forms may affect elderly persons, especially sporadic CJD. Sporadic CJD is a rare cause ... of dementia among middle-aged and elderly persons, and typical cases are clinically fairly distinct from more ... Creutzfeldt-Jakob disease (CJD) exists in inherited, acquired (variant and iatrogenic), and ...

    Abstract Creutzfeldt-Jakob disease (CJD) exists in inherited, acquired (variant and iatrogenic), and spontaneous (sporadic) forms. Although iatrogenic and variant forms of CJD usually affect relatively young persons, all forms may affect elderly persons, especially sporadic CJD. Sporadic CJD is a rare cause of dementia among middle-aged and elderly persons, and typical cases are clinically fairly distinct from more common forms of neurodegenerative dementias. However, clinical diagnosis can be a challenge for those who are not experienced with the disease. Fortunately, certain investigations can be very helpful. Although many cases of CJD (especially sporadic CJD) are not thought to be acquired illnesses, there is still a potential for onward transmission, and certain precautions are necessary to protect public health.
    MeSH term(s) Adolescent ; Adult ; Aged ; Aged, 80 and over ; Creutzfeldt-Jakob Syndrome/classification ; Creutzfeldt-Jakob Syndrome/complications ; Creutzfeldt-Jakob Syndrome/diagnosis ; Creutzfeldt-Jakob Syndrome/transmission ; Dementia/etiology ; Humans ; Middle Aged ; Prion Diseases
    Language English
    Publishing date 2006-08-01
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1099781-7
    ISSN 1537-6591 ; 1058-4838
    ISSN (online) 1537-6591
    ISSN 1058-4838
    DOI 10.1086/505215
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1505: Show issues Location:
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    Zs.MO 480: Show issues

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  2. Article: Report of a Case of Creutzfeldt-Jakob Disease With an Unusual Quick Evolution.

    Fadili, Hajar / Tazi, Rim / El Oury, Hiba / El Aidaoui, Karim / Hazim, Asmaa

    Cureus

    2022  Volume 14, Issue 3, Page(s) e22982

    Abstract: Creutzfeldt-Jakob disease is a rare, transmissible neurodegenerative disorder, most prevalent ... 3-3 protein was positive, which supports the diagnosis of Creutzfeldt-Jakob disease. Our observation ... between the ages of 50 and 70 years old, that is incurable and fatal. It's caused by a slow ...

    Abstract Creutzfeldt-Jakob disease is a rare, transmissible neurodegenerative disorder, most prevalent between the ages of 50 and 70 years old, that is incurable and fatal. It's caused by a slow, infectious protein agent-designated prion. The most common clinical presentations are sleep disturbances, personality changes, ataxia, aphasia, visual disturbances, weakness, and myoclonus combined with progressive dementia. Here we report the case of a patient with disturbance of consciousness, restlessness, and myoclonia who died two weeks after admission. The analysis of his cerebrospinal fluid reveals that the presence of 14-3-3 protein was positive, which supports the diagnosis of Creutzfeldt-Jakob disease. Our observation underscores the importance of the quick fatality of this case.
    Language English
    Publishing date 2022-03-09
    Publishing country United States
    Document type Case Reports
    ZDB-ID 2747273-5
    ISSN 2168-8184
    ISSN 2168-8184
    DOI 10.7759/cureus.22982
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Gerstmann-Sträussler-Scheinker syndrome with the P102L pathogenic mutation presenting as familial Creutzfeldt-Jakob disease: a case report and review of the literature.

    Rusina, Robert / Fiala, Jindřich / Holada, Karel / Matějčková, Milada / Nováková, Jana / Ampapa, Radek / Koukolík, František / Matěj, Radoslav

    Neurocase

    2013  Volume 19, Issue 1, Page(s) 41–53

    Abstract: ... magnetic resonance findings, which were highly suggestive of familial Creutzfeldt-Jakob disease. ... Gerstmann-Sträussler-Scheinker syndrome is a rare autosomal dominant disease caused by a mutation ... Sträussler-Scheinker syndrome by rapid progression, severe dementia, abnormal electroencephalography and ...

    Abstract Gerstmann-Sträussler-Scheinker syndrome is a rare autosomal dominant disease caused by a mutation in the prion gene, usually manifesting as progressive ataxia with late cognitive decline. A 44-year-old woman with a positive family history developed early personality and behavior changes, followed by paresthesias and ataxia, later associated with memory problems, pyramidal signs, anosognosia and very late myoclonus, spasticity, and severe dysexecutive impairment. Magnetic resonance showed caudate, mesio-frontal, and insular hyper-intensities, electroencephalography revealed generalized triphasic periodic complexes. A pathogenic P102L mutation in the prion gene was detected. Our case differed from classical Gerstmann-Sträussler-Scheinker syndrome by rapid progression, severe dementia, abnormal electroencephalography and magnetic resonance findings, which were highly suggestive of familial Creutzfeldt-Jakob disease.
    MeSH term(s) Adult ; Brain/pathology ; Creutzfeldt-Jakob Syndrome/genetics ; Creutzfeldt-Jakob Syndrome/psychology ; Diffusion Magnetic Resonance Imaging ; Electroencephalography ; Fatal Outcome ; Female ; Gait Disorders, Neurologic/etiology ; Gerstmann-Straussler-Scheinker Disease/genetics ; Gerstmann-Straussler-Scheinker Disease/psychology ; Humans ; Image Processing, Computer-Assisted ; Magnetic Resonance Imaging ; Memory/physiology ; Mutation/genetics ; Mutation/physiology ; Neuropsychological Tests ; Personality Disorders/etiology ; Personality Disorders/psychology ; Prions/genetics ; Psychomotor Performance/physiology ; Stroke/complications
    Chemical Substances Prions
    Language English
    Publishing date 2013
    Publishing country England
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1302651-3
    ISSN 1465-3656 ; 1355-4794
    ISSN (online) 1465-3656
    ISSN 1355-4794
    DOI 10.1080/13554794.2011.654215
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 4412: Show issues Location:
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