Miastenia grave

Última revisión: 1 Nov 2022
Última actualización: 30 Ago 2022

Resumen

Definición

Anamnesis y examen

Principales factores de diagnóstico

  • fuerza muscular fatiga
  • ptosis
  • diplopía
  • disfagia
  • disartría
  • paresia facial
  • debilidad en las extremidades proximales
  • disnea
Más principales factores de diagnóstico

Factores de riesgo

  • antecedentes familiares de trastornos autoinmunes
  • marcadores genéticos
  • tratamiento dirigido contra el cáncer
Más factores de riesgo

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

  • análisis sérico de anticuerpos frente a los receptores de acetilcolina (AChR)
  • anticuerpos frente a la tirosina cinasa específica del músculo (MuSK)
  • pruebas funcionales respiratorias en serie
Más primeras pruebas diagnósticas para solicitar

Pruebas diagnósticas que deben considerarse

  • ensayos de anticuerpos contra el receptor estriado
  • estimulación nerviosa repetitiva
  • electromiograma (EMG) de fibra aislada
  • tomografía computarizada (TC) de tórax
Más pruebas diagnósticas que deben considerarse

Algoritmo de tratamiento

Agudo

crisis miasténica

En curso

enfermedad de leve a moderada (clase I a III)

enfermedad grave (clase IV o V) o refractaria

Colaboradores

Autores

Robert P. Lisak, MD, FRCP (E), FAAN, FANA

Parker Webber Chair in Neurology

Professor of Neurology

Professor of Immunology and Microbiology

Wayne State University School of Medicine

Detroit

MI

Divulgaciones

RPL currently serves on an Advisory Board for Argenx for myasthenia gravis (MG), and was an Advisory Board member for Argenx (developing a clinical trial for chronic inflammatory demyelinating polyneuropathy [CIDP]). RPL has been reimbursed by Argenx the manufacturer of efartigimod for serving on a clinical advisory board. RPL has worked with Alexion (testing a treatment for MG); Novartis (design of research related to primary progressive multiple sclerosis [MS] vs. secondary progressive MS); Horizon Pharmaceuticals (advising on MG and neuromyelitis optica spectrum disorder); Takeda (advising on autoimmune diseases); GLG Consulting, Putnam Consulting, Slingshot Consulting, Haven Consulting, Clearview Consulting, Alpha Sites Consulting, and Insights Consulting (providing insights on the current and future treatments of several neuroimmunologic diseases including MS, MG, Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, and neuromyelitis optica spectrum disorder). RPL has presented several CME talks for companies with multiple sources of support (no direct payments to RPL). RPL has received clinical trial grants (no direct payments) for studies in MG (Alexion, Argenx, Ra Pharmaceuticals) and MS (Novartis, Teva, Genentech/Roche, Chugai). He has received royalties from Blackwell Press as an editor of the textbook International Neurology, and from Oxford University Press as an editor of a book, Neuroimmunology. RPL is an author of several papers cited in this topic.

Agradecimientos

Dr Robert Lisak would like to gratefully acknowledge Dr Andrea Corse and Dr Ami Mankodi, previous contributors to this topic.

Divulgaciones

AC and AM declare that they have no competing interests.

Revisores por pares

Vern C. Juel, MD

Associate Professor of Medicine (Neurology)

Duke University

Durham

NC

Divulgaciones

VCJ declares that he has no competing interests.

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