Isolated Chorea Associated with LGI1 Antibody

  • Ritesh A. Ramdhani
  • Steven J. Frucht

Abstract

Background: Leucine‐rich glioma inactivated 1 (LGI1) antibody produces a syndrome of limbic encephalitis, hyponatremia, and facio‐brachial dystonic seizures that is non‐paraneoplastic and responsive to corticosteroids. Parkinsonism, tremor, and generalized chorea are rare manifestations of LGI1, but, when present, commonly accompany other signs of limbic encephalitis.

Case Report: We present a case of LGI1‐related isolated chorea in a 53‐year‐old Japanese male. His chorea responded to high‐dose steroids, suggesting a potential role for this synaptic antibody in triggering chorea.

Discussion: This case highlights a new treatable etiology of chorea.

This article prompted a response letter:
Vynogradova I, Savitski V, Heckmann JG. Hemichorea associated with CASPR2 antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8VM49C5

The authors' response to the letter:
Ramdhani RA, Frucht SJ. Reply to: hemichorea associated with CASPR2 antibody. Tremor Other Hyperkinet Mov. 2014; 4. doi: 10.7916/D8QV3JM0