Constatations électrophysiologiques chez les patients MG positifs LRP4

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Pboulanger Prés.
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Constatations électrophysiologiques chez les patients MG positifs LRP4

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Electrophysiological findings in patients with low density lipoprotein receptor related protein 4 positive myasthenia gravis

A. V. Nikolic1,2,*, S. D. Bojic3, V. M. Rakocevic Stojanovic1,2, I. Z. Basta1,2 andD. V. Lavrnic1,2
Version of Record online: 14 JUL 2016
DOI: 10.1111/ene.13081


  • Background and purpose

    The aim was to determine the electrophysiological profile of our cohort of low density lipoprotein receptor related protein 4 (LRP4) positive myasthenia gravis (MG) patients.

  • Methods
    A repetitive nerve stimulation (RNS) test and jitter analysis using a concentric needle electrode were performed in 17 LRP4 positive MG patients. The results were compared to 31 muscle-specific tyrosine kinase (MuSK) positive and 28 acetylcholine receptor (AChR) positive MG patients.


  • Results
    The RNS test was negative in almost all patients belonging to the LRP4/seronegative and LRP4/MuSK groups. It was positive most frequently in the AChR MG patients, especially those without anti-LRP4 antibodies. The presence of anti-LRP4 antibodies was connected to lower decrement values, whilst the independent presence of anti-AChR or anti-MuSK antibodies was connected to higher decrement values. Lowest jitter was recorded in patients with LRP4/seronegative MG. The highest percentage of pathological jitter analysis test results was present in MuSK and AChR MG patients. The isolate presence of anti-LRP4 antibodies did not influence the mean consecutive difference values, whilst mean consecutive difference values were higher in the presence of anti-AChR or anti-MuSK antibodies.

  • Conclusions
    Low density lipoprotein receptor related protein 4 positive patients make a distinct MG subgroup with rarely detected pathological electrophysiological test results. The lack of influence of anti-LRP4 antibodies on the different electrophysiological parameters brings into question the pathogenic role of anti-LRP4 antibodies in MG.

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