შეჯამება
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- fraqueza nos membros
- xerostomia
- fraqueza
Other diagnostic factors
- disartria
- arreflexia
- ptose
- diplopia
- impotência
- disfagia
- hipotensão ortostática
- dilatação pupilar
- dispneia
- ataxia cerebelar
Risk factors
- câncer pulmonar de células pequenas subjacente ou outra malignidade
- distúrbio autoimune coexistente
- tabagismo
- história familiar de doença autoimune
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)
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)
Emerging tests
- anticorpos antissubunidade dos canais de cálcio dependentes de voltagem alfa-1A P/Q
- anticorpos anti-SOX1
Treatment algorithm
fraqueza respiratória ou bulbar intensa
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
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.
Differentials
- Botulismo
- Miastenia gravis
- Miopatia
More DifferentialsGuidelines
- 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 GuidelinesFolhetos informativos para os pacientes
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