Triple-seronegative myasthenia gravis: clinical and epidemiological characteristics

Abstract: Background Myasthenia gravis (MG) is an autoimmune disease usually caused by antibodies against the acetylcholine receptor (AChR-Abs), muscle-specific tyrosine kinase (MuSK-Abs), or low-density lipoprotein receptor-related protein 4 (LRP4-Abs). However, there are MG patients who do not have these antibodies and are thus said to have triple-seronegative (triple-SN) MG. Objective This study aims to describe the frequency and clinical and epidemiological characteristics of patients with triple-SN MG. Methods This was a retrospective cross-sectional study carried out through the analysis of medical records. Descriptive and analytical statistical analysis was performed comparing subgroups of myasthenic patients, classified according to serological profile. Results The sample population consisted of 93 MG patients: 85 were positive for antibodies, 80 (86%) with AChR-Abs, 5 (5.4%) with MuSK-Abs, and no MG patients with LRP4-Abs. Eight patients (8.6%) had triple-SN MG; they had a median age at disease onset of 30 years (21-45). Their most common initial symptoms were ptosis, diplopia, and generalized weakness. Most patients presented with mild symptoms at their last visit, reflecting a median MG composite scale score of 4 (0-6), and 75% of patients had an adequate response to treatment. Conclusion Our study showed a low frequency of triple-SN MG in Brazilian MG patients. Triple-SN MG was predominant in females, who presented with ptosis, diplopia, and generalized weakness, and most patients had an adequate response to immunosuppressive treatment. There was no significant difference between triple-SN MG and the other subgroups.

Alternative title
Miastenia gravis triplo-soronegativa: características clínicas e epidemiológicas
Location
Deutsche Nationalbibliothek Frankfurt am Main
Extent
Online-Ressource
Language
Englisch

Bibliographic citation
Triple-seronegative myasthenia gravis: clinical and epidemiological characteristics ; volume:82 ; number:01 ; year:2024 ; pages:001-007
Arquivos de neuro-psiquiatria ; 82, Heft 01 (2024), 001-007

Contributor
Rodrigues, Paula Raquel do Vale Pascoal
Kay, Cláudia Suemi Kamoi
Ducci, Renata Dal-Pra
Utiumi, Marco Antonio Takashi
Fustes, Otto Jesus Hernandez
Werneck, Lineu Cesar
Lorenzoni, Paulo José
Scola, Rosana Herminia

DOI
10.1055/s-0044-1779052
URN
urn:nbn:de:101:1-2404181101108.993108317482
Rights
Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
Last update
14.08.2025, 10:47 AM CEST

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Associated

  • Rodrigues, Paula Raquel do Vale Pascoal
  • Kay, Cláudia Suemi Kamoi
  • Ducci, Renata Dal-Pra
  • Utiumi, Marco Antonio Takashi
  • Fustes, Otto Jesus Hernandez
  • Werneck, Lineu Cesar
  • Lorenzoni, Paulo José
  • Scola, Rosana Herminia

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