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Objective <p>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.</p> Methods <p>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.</p> Results <p>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&#xa0;mg, once a week for 4&#xa0;times), and the symptoms were almost alleviated at 6&#xa0;months follow-up. The patient 2 was a 31&#xa0;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&#xa0;mg, once a week for 4&#xa0;times), and follow-up at 6&#xa0;months also showed recovery of muscle strength.</p> Conclusion <p>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.</p>

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Successful treatment of morvan syndrome with efgartigimod: report of two cases

  • 期刊论文

  • 发表日期：

    2025年4月23日

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  • Peng Wang,
  • Pengpeng Li,
  • Ruojie He,
  • Xiaoli Yao

摘要

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.