Evidence map›Paper›PMID 32907971›Full record

ArticleNeurology2020

Determinants of the repetitive-CMAP occurrence and therapy efficacy in slow-channel myasthenia.

Li Di, Hai Chen, Yan Lu, Duygu Selcen, Andrew G Engel, Yuwei Da, Xin-Ming Shen

Open access · greenAbstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
0.6field-weighted citation impact, top 27% 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

4 citing papers in PubMed, 13 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors at 1 institution in 1 country.

Li DiFrom the Department of Neurology (L.D., H.C., Y.L., Y.D.), Xuanwu Hospital, Capital Medical University, Beijing, China; and Department of Neurology and Neuromuscular Research Laboratory (L.D., D.S., A.G.E., X.-M.S.), Mayo Clinic, Rochester, MN.
Hai ChenFrom the Department of Neurology (L.D., H.C., Y.L., Y.D.), Xuanwu Hospital, Capital Medical University, Beijing, China; and Department of Neurology and Neuromuscular Research Laboratory (L.D., D.S., A.G.E., X.-M.S.), Mayo Clinic, Rochester, MN.
Yan LuFrom the Department of Neurology (L.D., H.C., Y.L., Y.D.), Xuanwu Hospital, Capital Medical University, Beijing, China; and Department of Neurology and Neuromuscular Research Laboratory (L.D., D.S., A.G.E., X.-M.S.), Mayo Clinic, Rochester, MN.
Duygu SelcenFrom the Department of Neurology (L.D., H.C., Y.L., Y.D.), Xuanwu Hospital, Capital Medical University, Beijing, China; and Department of Neurology and Neuromuscular Research Laboratory (L.D., D.S., A.G.E., X.-M.S.), Mayo Clinic, Rochester, MN.
Andrew G EngelFrom the Department of Neurology (L.D., H.C., Y.L., Y.D.), Xuanwu Hospital, Capital Medical University, Beijing, China; and Department of Neurology and Neuromuscular Research Laboratory (L.D., D.S., A.G.E., X.-M.S.), Mayo Clinic, Rochester, MN.
Yuwei DaFrom the Department of Neurology (L.D., H.C., Y.L., Y.D.), Xuanwu Hospital, Capital Medical University, Beijing, China; and Department of Neurology and Neuromuscular Research Laboratory (L.D., D.S., A.G.E., X.-M.S.), Mayo Clinic, Rochester, MN. dayuwei100@hotmail.com shen.xinming@mayo.edu.
Xin-Ming ShenFrom the Department of Neurology (L.D., H.C., Y.L., Y.D.), Xuanwu Hospital, Capital Medical University, Beijing, China; and Department of Neurology and Neuromuscular Research Laboratory (L.D., D.S., A.G.E., X.-M.S.), Mayo Clinic, Rochester, MN. dayuwei100@hotmail.com shen.xinming@mayo.edu.ORCID 0000-0002-7087-5166
Capital Medical University · CN

Funding

Congenital Myasthenic SyndromesR01NS109491 · NINDS · MAYO CLINIC ROCHESTER · PI ENGEL, ANDREW GEORGE, SELCEN, DUYGU · 2019 to 2022
$1.8M
NINDS NIH HHS R01 NS109491
6 · The paper itself

Abstract

objectiveTo find determinants of the occurrence of repetitive compound muscle action potential (R-CMAP) and to assess the efficacy of channel blocker therapy in slow-channel congenital myasthenic syndrome (SCCMS).

methodsNeurologic examination, EMG study, laboratory test, muscle biopsy, and next-generation and Sanger sequencing; literature review of reported patients with SCCMS, including EMG, kinetics of mutant acetylcholine receptors (AChRs), and response to therapy; and simulation of the decay phase of endplate potential (EPP) were performed.

resultsThree newly characterized and 57 reported patients with SCCMS with mutations of AChR subunits were included. In patients with R-CMAP, the length of channel opening bursts of mutant AChR was increased 8.68 ± 2.82 (mean ± SD)-fold compared to wild-type; in patients without R-CMAP, the length was increased 3.84 ± 0.65-fold (95% confidence interval 3.18-6.50,

conclusionsIn SCCMS, the R-CMAP occurrence is related to the extent of prolongation of the opening episodes of mutant AChR channel. Channel blocker treatment is more effective the sooner it is started after the onset of symptoms. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that channel blocker therapy in patients with SCCMS improves symptoms.

Indexed as

AdultCholinesterase InhibitorsElectromyographyFemaleHumansMembrane Transport ModulatorsMiddle AgedMuscle, SkeletalMyasthenic Syndromes, CongenitalPedigreePyridostigmine BromideReceptors, CholinergicYoung AdultCholinesterase InhibitorsMembrane Transport ModulatorsPyridostigmine BromideReceptors, Cholinergic

Identifiers

PMID32907971
PMCPMC7734725
OpenAlexW3085893709

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.