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Anti-LGI1–Associated Myopathy in the Setting of Neuromuscular Hyperexcitability Syndrome

To identify the key insights or developments described in this article
1 Credit CME

Anti–leucine-rich glioma-inactivated 1 (LGI1) antibodies are typically associated with autoimmune encephalitis; however, the clinical spectrum is evolving. Peripheral neuropathy and neuromuscular hyperexcitability syndrome have recently been recognized.13 We describe a case of anti-LGI1–associated hyperexcitability syndrome associated with myopathy.

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Article Information

Corresponding Author: Peter Anderyas, MBBS, St Vincent’s Hospital Melbourne, 41 Victoria Parade, Fitzroy, VIC 3065, Australia (peter.anderyas@svha.org.au).

Published Online: October 24, 2022. doi:10.1001/jamaneurol.2022.3479

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We also acknowledge and thank Penny McKelvie, MBBS (St Vincent’s Hospital Melbourne), for providing pathology images and interpretation; compensation was not received.

References
1.
Sophie  NMB , Klein  CJ , Waters  P , Pittock  SJ , Irani  SR .  LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes.   J Neurol Neurosurg Psychiatry. 2018;89(5):526-534. doi:10.1136/jnnp-2017-315720Google ScholarCrossref
2.
Irani  SR , Alexander  S , Waters  P ,  et al.  Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia.   Brain. 2010;133(9):2734-2748. doi:10.1093/brain/awq213PubMedGoogle ScholarCrossref
3.
Gadoth  A , Pittock  SJ , Dubey  D ,  et al.  Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG-positive patients.   Ann Neurol. 2017;82(1):79-92. doi:10.1002/ana.24979PubMedGoogle ScholarCrossref
AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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