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  1. Article: Arginine for the Treatment of Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes: A Systematic Review.

    Argudo, Jennifer M / Astudillo Moncayo, Olga M / Insuasti, Walter / Garofalo, Gabriela / Aguirre, Alex S / Encalada, Sebastian / Villamarin, Jose / Oña, Sebastian / Tenemaza, Maria Gabriela / Eissa-Garcés, Ahmed / Matcheswalla, Sakina / Ortiz, Juan Fernando

    Cureus

    2022  Volume 14, Issue 12, Page(s) e32709

    Abstract: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a mitochondrial disease that lacks a definitive treatment. Lately, there has been an increased interest in the scientific community about the role of arginine in ... ...

    Abstract Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a mitochondrial disease that lacks a definitive treatment. Lately, there has been an increased interest in the scientific community about the role of arginine in the short and long-term settings of the disease. We aim to conduct a systematic review of the clinical use of arginine in the management of MELAS and explore the role of arginine in the pathophysiology of the disease. We used PubMed advanced-strategy searches and only included full-text clinical trials on humans written in the English language. After applying the inclusion/exclusion criteria, four clinical trials were reviewed. We used the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guidelines and Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol for this systematic review. We used the Cochrane Collaboration risk-of-bias tool to assess the bias encountered in each study. Overall, IV arginine seems to be effective in improving symptoms during acute attacks of MELAS, while oral arginine supplementation increases endothelial function, preventing further stroke-like episodes.
    Language English
    Publishing date 2022-12-19
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2747273-5
    ISSN 2168-8184
    ISSN 2168-8184
    DOI 10.7759/cureus.32709
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: The Alien Limb Phenomenon in Creutzfeldt-Jakob Disease: A Systematic Review of Case Reports.

    Sen, Meghdeep / Moncayo, Juan A / Kelley, Maria A / Suarez Salazar, Deydie / Tenemaza, Maria Gabriela / Camacho, Mikaela / Hassen, Gashaw / Lopez, Guillermo E / Monteros, Gustavo / Garofalo, Gabriela / Yadav, Ayush / Ortiz, Juan Fernando

    Cureus

    2022  Volume 14, Issue 7, Page(s) e27029

    Abstract: Alien limb phenomenon (ALP) is a clinical finding seen in numerous neurological disorders, including Creutzfeldt-Jakob disease (CJD). We aimed to conduct a systematic review to update advances in understanding the classification and pathophysiology of ... ...

    Abstract Alien limb phenomenon (ALP) is a clinical finding seen in numerous neurological disorders, including Creutzfeldt-Jakob disease (CJD). We aimed to conduct a systematic review to update advances in understanding the classification and pathophysiology of ALP in CJD. We used PubMed advanced-strategy searches and only included full-text observational studies and case reports conducted on humans and written in English. We used the PRISMA protocol for this systematic review and the Methodological Quality of Case Reports tool to assess the bias encountered in each study. After applying the inclusion/exclusion criteria, 10 case reports were reviewed. Two independent reviewers analyzed data and confirmed the phenotype of each case of the alien limb in CJD separately. Overall, the most prevalent ALP phenotype presenting in patients with CJD was the posterior phenotype, usually in the early stages of the disease. Our findings corroborate previous research in demonstrating the pathophysiology behind ALP in CJD. We suggest physicians suspect CJD whenever patients present with ALP as the initial symptom.
    Language English
    Publishing date 2022-07-19
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2747273-5
    ISSN 2168-8184
    ISSN 2168-8184
    DOI 10.7759/cureus.27029
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Relevance of Fluorodopa PET Scan in Dopamine Responsive Dystonia and Juvenile Parkinsonism: A Systematic Review.

    Moncayo, Juan A / Vargas, Maite / Ortiz, Juan F / Granda, Pablo / Aguirre, Alex S / Argudo, Jennifer / Tambo, Willians / Garofalo, Gabriela / Capirig, Christian John / German-Montenegro, Melisa / Rueda, Luis G

    Neurology international

    2022  Volume 14, Issue 4, Page(s) 997–1006

    Abstract: Background: Dopamine Responsive Dystonia (DRD) and Juvenile Parkinsonism (JP) are two diseases commonly presenting with parkinsonian symptoms in young patients. Current clinical guidelines offer a diagnostic approach based on molecular analysis. However, ...

    Abstract Background: Dopamine Responsive Dystonia (DRD) and Juvenile Parkinsonism (JP) are two diseases commonly presenting with parkinsonian symptoms in young patients. Current clinical guidelines offer a diagnostic approach based on molecular analysis. However, developing countries have limitations in terms of accessibility to these tests. We aimed to assess the utility of imaging equipment, usually more available worldwide, to help diagnose and improve patients' quality of life with these diseases.
    Methods: We performed a systematic literature review in English using the preferred reporting items for systematic reviews and meta-analyses (PRISMA) and meta-analysis of observational studies in epidemiology (MOOSE) protocols. We only used human clinical trials about dopamine responsive dystonia and juvenile parkinsonism patients in which a fluorodopa (FD) positron emission tomography (PET) scan was performed to identify its use in these diseases.
    Results: We included six studies that fulfilled our criteria. We found a clear pattern of decreased uptake in the putamen and caudate nucleus in JP cases. At the same time, the results in DRD were comparable to normal subjects, with only a slightly decreased marker uptake in the previously mentioned regions by the FD PET scan.
    Conclusions: We found a distinctive pattern for each of these diseases. Identifying these findings with FD PET scans can shorten the delay in making a definitive diagnosis when genetic testing is unavailable, a common scenario in developing countries.
    Language English
    Publishing date 2022-12-02
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2514727-4
    ISSN 2035-8377 ; 2035-8385
    ISSN (online) 2035-8377
    ISSN 2035-8385
    DOI 10.3390/neurolint14040079
    Database MEDical Literature Analysis and Retrieval System OnLINE

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