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  1. Article ; Online: Inflammatory myopathies: an update for neurologists.

    Silva, André Macedo Serafim / Campos, Eliene Dutra / Zanoteli, Edmar

    Arquivos de neuro-psiquiatria

    2022  Volume 80, Issue 5 Suppl 1, Page(s) 238–248

    Abstract: Idiopathic inflammatory myopathies (IIM) are a heterogenous group of treatable myopathies. Patients present mainly to the rheumatologist and neurologists, complaining of acute or subacute onset of proximal weakness. Extramuscular manifestations may occur, ...

    Abstract Idiopathic inflammatory myopathies (IIM) are a heterogenous group of treatable myopathies. Patients present mainly to the rheumatologist and neurologists, complaining of acute or subacute onset of proximal weakness. Extramuscular manifestations may occur, including involvement of the lungs, skin, and joints. Classically, the diagnosis used to be made based on the creatine kinase level increase, abnormalities in electroneuromyography and presence of inflammatory infiltrates in the muscle biopsy. Recently, the importance of autoantibodies has increased, and now they may be identified in more than half of IIM patients. The continuous clinicoseropathological improvement in IIM knowledge has changed the way we see these patients and how we classify them. In the past, only polymyositis, dermatomyositis and inclusion body myopathy were described. Currently, immune-mediated necrotizing myopathy, overlap myositis and antisynthetase syndrome have been considered the most common forms of IIM in clinical practice, increasing the spectrum of classification. Patients previously considered to have polymyositis, in fact have these other forms of seropositive IIM. In this article, we reviewed the new concepts of classification, a practical way to make the diagnosis and how to plan the treatment of patients suffering from IIM.
    MeSH term(s) Autoantibodies ; Dermatomyositis/diagnosis ; Dermatomyositis/pathology ; Humans ; Muscular Diseases ; Myositis/diagnosis ; Myositis/therapy ; Neurologists ; Polymyositis
    Chemical Substances Autoantibodies
    Language English
    Publishing date 2022-08-17
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 418916-4
    ISSN 1678-4227 ; 0004-282X
    ISSN (online) 1678-4227
    ISSN 0004-282X
    DOI 10.1590/0004-282X-ANP-2022-S131
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Inflammatory myopathies: an update for neurologists

    Silva, André Macedo Serafim / Campos, Eliene Dutra / Zanoteli, Edmar

    Arquivos de Neuro-Psiquiatria

    2022  Volume 80, Issue S 05, Page(s) 238–248

    Abstract: Idiopathic inflammatory myopathies (IIM) are a heterogenous group of treatable myopathies. Patients present mainly to the rheumatologist and neurologists, complaining of acute or subacute onset of proximal weakness. Extramuscular manifestations may occur, ...

    Abstract Idiopathic inflammatory myopathies (IIM) are a heterogenous group of treatable myopathies. Patients present mainly to the rheumatologist and neurologists, complaining of acute or subacute onset of proximal weakness. Extramuscular manifestations may occur, including involvement of the lungs, skin, and joints. Classically, the diagnosis used to be made based on the creatine kinase level increase, abnormalities in electroneuromyography and presence of inflammatory infiltrates in the muscle biopsy. Recently, the importance of autoantibodies has increased, and now they may be identified in more than half of IIM patients. The continuous clinicoseropathological improvement in IIM knowledge has changed the way we see these patients and how we classify them. In the past, only polymyositis, dermatomyositis and inclusion body myopathy were described. Currently, immune-mediated necrotizing myopathy, overlap myositis and antisynthetase syndrome have been considered the most common forms of IIM in clinical practice, increasing the spectrum of classification. Patients previously considered to have polymyositis, in fact have these other forms of seropositive IIM. In this article, we reviewed the new concepts of classification, a practical way to make the diagnosis and how to plan the treatment of patients suffering from IIM.
    Keywords Myositis ; Dermatomyositis ; Polymyositis ; Immune-Mediated Necrotizing Myopathy ; Miosite ; Dermatomiosite ; Polimiosite ; Miopatia Necrosante Imunomediada
    Language English
    Publishing date 2022-05-01
    Publisher Thieme Revinter Publicações Ltda.
    Publishing place Stuttgart ; New York
    Document type Article
    ZDB-ID 418916-4
    ISSN 1678-4227 ; 0004-282X
    ISSN (online) 1678-4227
    ISSN 0004-282X
    DOI 10.1590/0004-282X-ANP-2022-S131
    Database Thieme publisher's database

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  3. Article ; Online: The Location of Disease-Causing DES Variants Determines the Severity of Phenotype and the Morphology of Sarcoplasmic Aggregates.

    Silva, André Macedo Serafim / Rodrigo, Patricia / Moreno, Cristiane Araújo Martins / Mendonça, Rodrigo de Holanda / Estephan, Eduardo de Paula / Camelo, Clara Gontijo / Campos, Eliene Dutra / Dias, Alexandre Torchio / Nascimento, Amom Mendes / Kulikowski, Leslie Domenici / Oliveira, Acary Souza Bulle / Reed, Umbertina Conti / Goldfarb, Lev G / Olivé, Montse / Zanoteli, Edmar

    Journal of neuropathology and experimental neurology

    2022  Volume 81, Issue 9, Page(s) 746–757

    Abstract: Desmin (DES) is the main intermediate muscle filament that connects myofibrils individually and with the nucleus, sarcolemma, and organelles. Pathogenic variants of DES cause desminopathy, a disorder affecting the heart and skeletal muscles. We aimed to ... ...

    Abstract Desmin (DES) is the main intermediate muscle filament that connects myofibrils individually and with the nucleus, sarcolemma, and organelles. Pathogenic variants of DES cause desminopathy, a disorder affecting the heart and skeletal muscles. We aimed to analyze the clinical features, morphology, and distribution of desmin aggregates in skeletal muscle biopsies of patients with desminopathy and to correlate these findings with the type and location of disease-causing DES variants. This retrospective study included 30 patients from 20 families with molecularly confirmed desminopathy from 2 neuromuscular referral centers. We identified 2 distinct patterns of desmin aggregates: well-demarcated subsarcolemmal aggregates and diffuse aggregates with poorly delimited borders. Pathogenic variants located in the 1B segment and the tail domain of the desmin molecule are more likely to present with early-onset cardiomyopathy compared to patients with variants in other segments. All patients with mutations in the 1B segment had well-demarcated subsarcolemmal aggregates, but none of the patients with variants in other desmin segments showed such histological features. We suggest that variants located in the 1B segment lead to well-shaped subsarcolemmal desmin aggregation and cause disease with more frequent cardiac manifestations. These findings will facilitate early identification of patients with potentially severe cardiac syndromes.
    MeSH term(s) Cardiomyopathies/genetics ; Cardiomyopathies/pathology ; Desmin/genetics ; Humans ; Muscle, Skeletal/pathology ; Mutation/genetics ; Phenotype ; Retrospective Studies
    Chemical Substances Desmin
    Language English
    Publishing date 2022-09-07
    Publishing country England
    Document type Journal Article
    ZDB-ID 3088-0
    ISSN 1554-6578 ; 0022-3069
    ISSN (online) 1554-6578
    ISSN 0022-3069
    DOI 10.1093/jnen/nlac063
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Histological changes underlying bupivacaine's effect on extra ocular muscle.

    Hopker, Luisa Moreira / Neves, Juliana de Carvalho / Nascimento, Daiane Jaqueline / Campos, Eliene Dutra / Mendonça, Tomas Scalamandre / Zanoteli, Edmar / Allemann, Norma

    Experimental eye research

    2018  Volume 171, Page(s) 62–67

    Abstract: To determine the changes in the cross-sectional area (CSA) of myofibers and their subtype distribution based on the myosin isoform expression after bupivacaine (BUP) injection in the EOM of rabbits and help the understanding of strabismus correction ... ...

    Abstract To determine the changes in the cross-sectional area (CSA) of myofibers and their subtype distribution based on the myosin isoform expression after bupivacaine (BUP) injection in the EOM of rabbits and help the understanding of strabismus correction after BUP injection in the clinical practice. A total of 32 rabbits received 0.3 mL of 1.5% BUP in the superior rectus muscle (SR) of the right eye (OD) and were sacrificed at days 7, 28, 60, and 92. Additional eight untouched rabbits were included as controls. Hematoxylin and eosin staining was performed, and ImageJ software was used to measure CSA. Immunohistochemical analysis was performed to analyze the proportion of myofibers positive for myosin types 1 (slow), 2 (fast) and embryonic. Myofiber area measurement decreased 7 days after BUP injection [SR, 1271 ± 412 μm
    MeSH term(s) Anesthetics, Local/pharmacology ; Animals ; Bupivacaine/pharmacology ; Collagen/metabolism ; Immunohistochemistry ; Injections, Intramuscular ; Male ; Muscle Fibers, Skeletal/drug effects ; Muscle Fibers, Skeletal/metabolism ; Muscle Fibers, Skeletal/pathology ; Myosin Type I/metabolism ; Myosin Type II/metabolism ; Oculomotor Muscles/drug effects ; Oculomotor Muscles/metabolism ; Oculomotor Muscles/pathology ; Protein Isoforms/metabolism ; Rabbits
    Chemical Substances Anesthetics, Local ; Protein Isoforms ; Collagen (9007-34-5) ; Myosin Type I (EC 3.6.1.-) ; Myosin Type II (EC 3.6.1.-) ; Bupivacaine (Y8335394RO)
    Language English
    Publishing date 2018-03-09
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 80122-7
    ISSN 1096-0007 ; 0014-4835
    ISSN (online) 1096-0007
    ISSN 0014-4835
    DOI 10.1016/j.exer.2018.03.004
    Database MEDical Literature Analysis and Retrieval System OnLINE

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