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Síndrome miastênica de Lambert-Eaton

ბოლო მიმოხილვა: 23 Sep 2025
ბოლო განახლება: 21 Feb 2024

შეჯამება

Definition

History and exam

Key diagnostic factors

  • presença de fatores de risco
  • fraqueza nos membros
  • xerostomia
  • fraqueza
Full details

Other diagnostic factors

  • disartria
  • arreflexia
  • ptose
  • diplopia
  • impotência
  • disfagia
  • hipotensão ortostática
  • dilatação pupilar
  • dispneia
  • ataxia cerebelar
Full details

Risk factors

  • câncer pulmonar de células pequenas subjacente ou outra malignidade
  • distúrbio autoimune coexistente
  • tabagismo
  • história familiar de doença autoimune
Full details

Diagnostic investigations

1st investigations to order

  • estudos da condução nervosa
  • estimulação nervosa repetitiva de baixa frequência
  • sorologia para canais de cálcio dependentes de voltagem anti-P/Q
  • tomografia do tórax
  • sorologia para antirreceptor da acetilcolina (AChR)
  • hormônio estimulante da tireoide (TSH)
Full details

Investigations to consider

  • testes de função pulmonar (TFPs) seriados
  • tomografia corporal total por emissão de pósitrons com flúor-2-deoxiglicose (FDG-PET)
  • broncoscopia
  • estimulação nervosa repetitiva (RNS) tetânica ou de alta frequência
  • eletromiografia de fibra única
  • haplótipos do antígeno leucocitário humano (HLA)
  • fatores antinucleares (FANs)
  • fator reumatoide (FR)
  • anticorpos anticitoplasma de neutrófilos (ANCA)
  • B12 e ácido metilmalônico (AMM)
Full details

Emerging tests

  • anticorpos antissubunidade dos canais de cálcio dependentes de voltagem alfa-1A P/Q
  • anticorpos anti-SOX1

Treatment algorithm

ACUTE

fraqueza respiratória ou bulbar intensa

ONGOING

sem fraqueza respiratória ou bulbar intensa

Contributors

Authors

Jonathan M. Morena, DO

Assistant Professor of Neurology

Duke University Medical Center

Durham

NC

Disclosures

JMM declares that he has no competing interests.

Joshua P. Alpers, MD

Staff Neurologist

University of Tennessee Erlanger Neurology

Chattanooga

TN

Disclosures

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

Disclosures

VCJ declares that he has no competing interests.

Peer reviewers

Olivia Tong, MD

Assistant Clinical Professor

UC Davis Medical Group

CA

Disclosures

OT declares that she has no competing interests.

Zaeem Siddiqui, MD, PhD

Associate Professor

Division of Neurology

University of Alberta

Edmonton

Canada

Disclosures

ZS declares that he has no competing interests.

Paul Wirtz, MD, PhD

Department of Neurology

Haga Hospital

The Hague

The Netherlands

Disclosures

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.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

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

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. Abstract

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.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
  • Síndrome miastênica de Lambert-Eaton images
  • Differentials

    • Botulismo
    • Miastenia gravis
    • Miopatia
    More Differentials
  • Guidelines

    • Updated consensus statement: intravenous immunoglobulin in the treatment of neuromuscular disorders report of the AANEM ad hoc committee
    • Clinical guidelines for immunoglobulin use (second edition update)
    More Guidelines
  • Folhetos informativos para os pacientes

    Abandono do hábito de fumar

    Mais Folhetos informativos para os pacientes
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