AUTHOR=Zhang Zunwei , Guan Yujia , Han Jiale , Li Mingming , Shi Miao , Deng Hui 

TITLE=Regional Features of MuSK Antibody-Positive Myasthenia Gravis in Northeast China

JOURNAL=Frontiers in Neurology

VOLUME=11

YEAR=2020

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2020.516211

DOI=10.3389/fneur.2020.516211

ISSN=1664-2295

ABSTRACT=<p><bold>Objective:</bold> To summarize the characteristics of muscle-specific receptor tyrosine kinase antibody-positive myasthenia gravis (MuSK-MG) in Northeast China.</p><p><bold>Methods:</bold> We retrospectively collected 183 confirmed MG patients and divided them into three groups based on the type of serum antibodies: MuSK-MG (14 cases), acetylcholine receptor (AChR)-MG (130 cases), and double-seronegative (DSN)-MG (39 cases). The clinical, diagnostic, therapeutic, and prognosis data were analyzed.</p><p><bold>Results:</bold> MuSK antibody was detected in 26.7% of seronegative MG. The mean age of onset in MuSK-MG was 53.2 ± 13.6 years. Fifty percent of MuSK-MG patients with an onset symptom of pure ocular muscle weakness. The time from onset to other muscle groups' involvement and the time from onset to myasthenic crisis had no significant difference among the three groups (<italic>P</italic> &gt; 0.05). The proportion of Osserman classification I in MuSK-MG group was lower than that in DSN-MG group. The proportion of Osserman classification IV in MuSK-MG group was higher than that in the other two groups. The incidences of other coexisting autoimmune diseases in MuSK-MG group were higher. Prognosis after the treatment of steroid combined with tacrolimus for MuSK-MG was similar to AChR-MG treated with steroid combined with an immunosuppressant agent (<italic>P</italic> &gt; 0.05).</p><p><bold>Conclusion:</bold> Patients with MuSK-MG in Northeast China have a modestly later onset age and a proportion of patients may have a mild form of the disease with delayed disease progression. We confirmed the existence of a rare ocular MuSK-MG phenotype, a high proportion of coexisting with other autoimmune diseases, and a good response to steroids combined with tacrolimus for our MuSK-MG series.</p>