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Eur J Neurol. 2016 Nov;23(11):1635-1641. doi: 10.1111/ene.13081. Epub 2016 Jul 14.

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

Author information

1
Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia. ananikolic74@yahoo.com.
2
Medical Faculty, Belgrade University, Belgrade, Serbia. ananikolic74@yahoo.com.
3
Institute for Medical Statistics, Medical Faculty, Belgrade University, Belgrade, Serbia.
4
Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia.
5
Medical Faculty, Belgrade University, Belgrade, Serbia.

Abstract

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.

KEYWORDS:

electrophysiology; jitter; low density lipoprotein-receptor-related protein 4; myasthenia gravis; neurophysiology; repetitive nerve stimulation

PMID:
27415961
DOI:
10.1111/ene.13081
[Indexed for MEDLINE]

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