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Successful treatment of morvan syndrome with efgartigimod: report of two cases
Peng Wang1,2, Pengpeng Li3, Ruojie He4
1Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases; National Key Clinical Department and Key Discipline of Neurology, Guangzhou, China.
Objective:
Morvan Syndrome is a rare autoimmune-mediated clinical condition. This retrospective study aims at assessing the effectiveness and safety of intravenous Efgartigimod in 2 cases of the Morvan Syndrome with positive LGI1 and CASPR2 antibodies.
Methods:
We reviewed the clinical manifestations, autoantibodies detection, electromyography (EMG) characteristics, treatments and follow-up outcomes in 2 patients of Morvan Syndrome with positive LGI1 and CASPR2 antibodies. Especially, we evaluated the therapeutic effectiveness and safety of intravenous Efgartigimod based on the improvement of symptoms, relapse conditions and adverse reactions.
Results:
The patient 1 was a 35-year-old female who presented with muscle tremors and pain in lower limbs. The serum VGKC, LGI1 and CASPR2 antibodies were positive. M-wave and muscle tremor discharges were observed in both gastrocnemius muscles with EMG examination. She was treated with efgartigimod (800 mg, once a week for 4 times), and the symptoms were almost alleviated at 6 months follow-up. The patient 2 was a 31 year-old female who presented with muscle soreness and weakness in four limbs. The serum VGKC, LGI1 and CASPR2 antibodies also were positive. M-wave discharges were observed with EMG examination. She was treated with efgartigimod (400 mg, once a week for 4 times), and follow-up at 6 months also showed recovery of muscle strength.
Conclusion:
Morvan Syndrome is an antibody-related autoimmune syndrome with complex clinical manifestations. Our findings showed that the treatments of intravenous Efgartigimod could be effective and well tolerant in patients of Morvan Syndrome with LGI1 and CASPR2 antibodies.
