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Lambert-Eaton myasthenic syndrome

Última revisión: 26 Aug 2025
Última actualización: 21 Feb 2024

Resumen

Definición

Anamnesis y examen

Principales factores de diagnóstico

  • history of small cell lung cancer or other malignancy
  • history of autoimmune disorder
  • history of cigarette smoking
  • family history of autoimmune disease
  • limb weakness
  • dry mouth
  • weakness
Todos los datos

Otros factores de diagnóstico

  • dysarthria
  • areflexia
  • ptosis
  • diplopia
  • impotence
  • dysphagia
  • orthostatic hypotension
  • pupillary dilation
  • dyspnea
  • cerebellar ataxia
Todos los datos

Factores de riesgo

  • underlying small cell lung cancer or other malignancy
  • coexisting autoimmune disorder
  • cigarette smoking
  • family history of autoimmune disease
Todos los datos

Pruebas diagnósticas

Primeras pruebas diagnósticas para solicitar

  • nerve conduction studies
  • low-frequency repetitive nerve stimulation
  • anti-P/Q voltage-gated calcium-channel serology
  • chest CT scan
  • anti-acetylcholine receptor (AChR) serology
  • thyroid-stimulating hormone (TSH)
Todos los datos

Pruebas diagnósticas que deben considerarse

  • serial PFTs
  • total-body fluoro-2-deoxyglucose positron emission tomography (FDG-PET) scan
  • bronchoscopy
  • high-frequency or tetanic repetitive nerve stimulation (RNS)
  • single-fiber electromyography
  • HLA haplotyping
  • antinuclear antibodies (ANA)
  • rheumatoid factor (RF)
  • antineutrophil cytoplasmic autoantibodies (ANCA)
  • B12 and methylmalonic acid (MMA)
Todos los datos

Pruebas emergentes

  • alpha-1A P/Q voltage-gated calcium-channel subunit antibodies
  • anti-SOX1 antibodies

Algoritmo de tratamiento

Agudo

severe respiratory or bulbar weakness

En curso

without severe respiratory or bulbar weakness

Colaboradores

Autores

Jonathan M. Morena, DO

Assistant Professor of Neurology

Duke University Medical Center

Durham

NC

Divulgaciones

JMM declares that he has no competing interests.

Joshua P. Alpers, MD

Staff Neurologist

University of Tennessee Erlanger Neurology

Chattanooga

TN

Divulgaciones

JA has been compensated by (1) Alexion, the manufacturer of Soliris and Ultomiris, (2) Argenx, the manufacturer of Vyvgart, and (3) MT Pharma, the manufacturer of Radicava ORS, for participation in advisory boards and speakers bureaus.

Vern C. Juel, MD

Professor of Neurology

Duke University Medical Center

Durham

NC

Declarações

VCJ declares that he has no competing interests.

Revisores

Olivia Tong, MD

Assistant Clinical Professor

UC Davis Medical Group

CA

Declarações

OT declares that she has no competing interests.

Zaeem Siddiqui, MD, PhD

Associate Professor

Division of Neurology

University of Alberta

Edmonton

Canada

Declarações

ZS declares that he has no competing interests.

Paul Wirtz, MD, PhD

Department of Neurology

Haga Hospital

The Hague

The Netherlands

Declarações

PW is an author of a number of references cited in this topic.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

Referências

Nossas equipes internas de editoria e de evidências trabalham em conjunto com colaboradores internacionais especializados e pares revisores para garantir que forneçamos acesso às informações o mais clinicamente relevantes possível.

Principais artigos

Titulaer MJ, Lang B, Verschuuren JJ. Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lancet Neurol. 2011 Dec;10(12):1098-107. Resumo

Skeie GO, Apostolski S, Evoli A, et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol. 2010 Jul;17(7):893-902. Resumo

AAEM Quality Assurance Committee, American Association of Electrodiagnostic Medicine. Practice parameter for repetitive nerve stimulation and single fiber EMG evaluation of adults with suspected myasthenia gravis or Lambert-Eaton myasthenic syndrome: summary statement. Muscle Nerve. 2001 Sep;24(9):1236-8.Texto completo  Resumo

Artigos de referência

Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
  • Lambert-Eaton myasthenic syndrome images
  • Diagnósticos diferenciais

    • Botulism
    • Myasthenia gravis
    • Myopathy
    Mais Diagnósticos diferenciais
  • Diretrizes

    • Updated consensus statement: intravenous immunoglobulin in the treatment of neuromuscular disorders report of the AANEM ad hoc committee
    • Screening for tumours in paraneoplastic syndromes: report of an EFNS task force
    Mais Diretrizes
  • Folhetos informativos para os pacientes

    Quitting smoking

    Mais Folhetos informativos para os pacientes
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