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Autoantibodies to Low-Density Lipoprotein Receptor-Related Protein 4 in Double Seronegative Myasthenia Gravis: A Systematic Review.
Bacchi, Stephen; Kramer, Philippe; Chalk, Colin.
Afiliação
  • Bacchi S; 1Adelaide Medical School,University of Adelaide,Adelaide,South Australia,Australia.
  • Kramer P; 2Faculty of Medicine,McGill University,Montreal,Quebec,Canada.
  • Chalk C; 3Department of Neurology & Neurosurgery,McGill University,Montreal,Quebec,Canada.
Can J Neurol Sci ; 45(1): 62-67, 2018 01.
Article em En | MEDLINE | ID: mdl-29334041
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which a clinical diagnosis may be confirmed with serological testing. The most common autoantibodies used to support a diagnosis of MG are anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies. In cases in which both of these autoantibodies are negative (termed double-seronegative [dSNMG]), other autoantibodies such as low-density lipoprotein receptor-related protein 4 (LRP4) may be used to aid in diagnosis. METHODS: We have undertaken a systematic literature review to identify studies that have assessed the frequency of anti-LRP4 antibodies in dSNMG patients and the characteristics of anti-LRP4+ dSNMG patients (epidemiology, clinical features, electromyographic findings, or management). PubMed, EMBASE, Medline, and Scopus were searched on January 14, 2017, using the medical subject headings "myasthenia gravis" and "low-density lipoprotein receptor-related protein 4" or "LRP4." RESULTS: The initial search identified 367 articles. Fourteen publications met the inclusion criteria. There were ten cross-sectional research studies, three were case series, and one was a case report. The majority of studies were limited by small sample sizes of LRP4+ dSNMG. There has been a wide range of frequencies of anti-LRP4 antibodies detected in different MG patient populations, some involving different laboratory techniques. CONCLUSIONS: LRP4+ dSNMG is more likely than LRP4- dSNMG to have a younger onset of disease and occur in females. LRP4+ dSNMG most often is mild in severity and often involves isolated ocular weakness. It typically responds well to pyridostigmine or prednisone.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Autoanticorpos / Proteínas Relacionadas a Receptor de LDL / Miastenia Gravis Idioma: En Ano de publicação: 2018 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Autoanticorpos / Proteínas Relacionadas a Receptor de LDL / Miastenia Gravis Idioma: En Ano de publicação: 2018 Tipo de documento: Article