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

2016 ◽  
Vol 23 (11) ◽  
pp. 1635-1641 ◽  
Author(s):  
A. V. Nikolic ◽  
S. D. Bojic ◽  
V. M. Rakocevic Stojanovic ◽  
I. Z. Basta ◽  
D. V. Lavrnic
Keyword(s):  
Myasthenia Gravis ◽  
Low Density Lipoprotein ◽  
Density Lipoprotein ◽  
Low Density ◽  
Lipoprotein Receptor ◽  
Related Protein ◽  
Low Density Lipoprotein Receptor ◽  
Density Lipoprotein Receptor ◽  
Electrophysiological Findings ◽  
Lipoprotein Receptor Related Protein

scholarly journals Autoantibodies to Low-Density Lipoprotein Receptor-Related Protein 4 in Double Seronegative Myasthenia Gravis: A Systematic Review

2017 ◽  
Vol 45 (1) ◽  
pp. 62-67 ◽  
Author(s):  
Stephen Bacchi ◽  
Philippe Kramer ◽  
Colin Chalk
Keyword(s):  
Myasthenia Gravis ◽  
Low Density Lipoprotein ◽  
Density Lipoprotein ◽  
Small Sample ◽  
Low Density ◽  
Lipoprotein Receptor ◽  
Related Protein ◽  
Low Density Lipoprotein Receptor ◽  
Density Lipoprotein Receptor ◽  
Lipoprotein Receptor Related Protein

AbstractBackground: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction in which a clinical diagnosis may be confirmed with serological testing. The most common autoantibodies used to support a diagnosis of MG are anti-acetylcholine receptor antibodies and anti-muscle-specific tyrosine kinase antibodies. In cases in which both of these autoantibodies are negative (termed double-seronegative [dSNMG]), other autoantibodies such as low-density lipoprotein receptor-related protein 4 (LRP4) may be used to aid in diagnosis. Methods: We have undertaken a systematic literature review to identify studies that have assessed the frequency of anti-LRP4 antibodies in dSNMG patients and the characteristics of anti-LRP4+ dSNMG patients (epidemiology, clinical features, electromyographic findings, or management). PubMed, EMBASE, Medline, and Scopus were searched on January 14, 2017, using the medical subject headings “myasthenia gravis” and “low-density lipoprotein receptor-related protein 4” or “LRP4.” Results: The initial search identified 367 articles. Fourteen publications met the inclusion criteria. There were ten cross-sectional research studies, three were case series, and one was a case report. The majority of studies were limited by small sample sizes of LRP4+ dSNMG. There has been a wide range of frequencies of anti-LRP4 antibodies detected in different MG patient populations, some involving different laboratory techniques. Conclusions: LRP4+ dSNMG is more likely than LRP4– dSNMG to have a younger onset of disease and occur in females. LRP4+ dSNMG most often is mild in severity and often involves isolated ocular weakness. It typically responds well to pyridostigmine or prednisone.

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