Evidence map›Paper›PMID 28608304›Full record

ReviewJournal of neurology2017

Lambert-Eaton myasthenic syndrome (LEMS): a rare autoimmune presynaptic disorder often associated with cancer.

Benedikt Schoser, Bruno Eymard, Joe Datt, Renato Mantegazza

Erratum issuedAbstract readReview
PubMed Publisher
In one paragraph

Review in Journal of neurology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 32 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
32citing papers in PubMed, 1 pooled it
6.1field-weighted citation impact, top 3% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

32 citing papers in PubMed, 1 synthesis or guideline pooled it, 84 citations in OpenAlex.

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  17. Paraneoplastic Syndromes in Neuro-Ophthalmology.Annals of Indian Academy of Neurology · 2022
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  20. Paraneoplastic neurological syndrome: growing spectrum and relevance.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022
    Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

4 authors at 4 institutions in 4 countries.

Benedikt SchoserFriedrich-Baur-Institute, Department of Neurology Klinikum München Ludwig-Maximilians-University Munich, Munich, Germany. bschoser@med.uni-muenchen.de.ORCID http://orcid.org/0000-0002-2757-8131
Bruno EymardInstitute de Myologie, 47 Boulevard de l'Hôpital, 75013, Paris, France.
Joe DattBioMarin Europe Ltd, 10 Bloomsbury Way, London, WC1A 2SL, UK.
Renato MantegazzaFondazione IRCCS Istituto Neurologico, Via Giovanni Celoria, 11, 20133, Milan, Italy.
BioMarin (United Kingdom) · GBFondazione IRCCS Istituto Neurologico Carlo Besta · ITInstitut de Myologie · FRLudwig-Maximilians-Universität München · DE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune neuromuscular junction disorder that is related to the loss of functional P/Q-type voltage-gated calcium channels (VGCCs) on presynaptic nerve terminals. Up to 60% of cases occur as a paraneoplastic disorder (SCLC-LEMS), most commonly in association with small cell lung cancer. The remaining cases have an idiopathic non-tumor etiology but are associated with underlying autoimmune disease (NT-LEMS). Patients with LEMS invariably experience progressive proximal muscle weakness, often accompanied by general fatigue and autonomic symptoms. Some LEMS clinical symptoms overlap with those of other myasthenic syndromes, most commonly myasthenia gravis, which can contribute to misdiagnosis or delayed diagnosis. Prognosis is related to the presence of cancer or autoimmune disease and the severity/distribution of muscle weakness. Cause of death in patients with SCLC-LEMS is typically tumor progression, whereas NT-LEMS does not reduce life expectancy. LEMS diagnosis is supported by a threefold approach: clinical features, electromyography, and anti-VGCC antibody serology. LEMS is a clinically important early indicator of possible cancer; therefore, a LEMS diagnosis should immediately prompt rigorous oncological screening and surveillance. Symptomatic treatment of LEMS typically involves medications that improve neurotransmission (e.g., the potassium channel blocker amifampridine [3,4-diaminopyridine]), with addition of immunosuppressants/modulators (e.g., prednisone plus azathioprine) in individuals with persistent symptoms. Where a tumor is identified, oncological treatment should take priority. It should be remembered, however, that LEMS has a significant impact on a patient's quality of life and ability to perform daily activities, and therefore warrants timely diagnosis and appropriate treatment in and of itself.

Indexed as

Calcium ChannelsHumansLambert-Eaton Myasthenic SyndromeLung NeoplasmsQuality of LifeRisk FactorsSmall Cell Lung CarcinomaCalcium ChannelsAutoimmunityLambert–Eaton myasthenic syndromeNeuromuscular junctionQuality of lifeSmall cell lung carcinomaVoltage-gated calcium channels

Identifiers

PMID28608304
OpenAlexW2626918921

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.