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Reply to: Hemichorea Associated with CASPR2 Antibody


Ritesh A. Ramdhani ,

Movement Disorders Division, Icahn School of Medicine at Mount Sinai, New York, New York, US
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Steven J. Frucht

Movement Disorders Division, Icahn School of Medicine at Mount Sinai, New York, New York, US
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How to Cite: Ramdhani RA, Frucht SJ. Reply to: Hemichorea Associated with CASPR2 Antibody. Tremor and Other Hyperkinetic Movements. 2014;4:246. DOI:
  Published on 19 May 2014
 Accepted on 01 May 2014            Submitted on 17 Apr 2014

To the editor:

The case presented by Vynogradova and colleagues, adds to the ever-expanding etiological spectrum of autoimmune chorea. This is the first reported case of isolated paraneoplastic chorea associated with anti-Caspr2, paralleling our report of isolated idiopathic chorea associated with anti-LGI1. Both cases share an asymmetric predominance of the chorea as well as the absence of limbic encephalitis. LGI1 and Caspr2 antibodies are specific to the VGKC-protein complex and each are associated with a number of distinct clinical phenotypes: Faciobrachial dystonic seizures (FBDS), hyponatremia and limbic encephalitis (LE) in the former; and Morvan’s syndrome1, peripheral nerve excitability, and Isaac’s syndrome2 in the latter. Despite their phenotypic differences, these two cases highlight that phenotypically-similar late-onset isolated chorea can be a result of distinct pathophysiological mechanisms which will result in a different clinical course. This is illustrated by the fact that, five months after having a complete remission following pulse steroid therapy, our patient’s chorea relapsed. A second course of steroids did not engender as robust a response as the initial treatment. Seizure, metabolic disturbances, and LE remain absent and a steroid-sparing therapy (i.e., rituximab, IVIG) will be considered next. These cases emphasize the importance of antibody screening in late-onset chorea due to the diagnostic and treatment implications.


1 Funding: None. 

2 Financial Disclosures: None. 

3 Conflict of Interests: The authors report no conflict of interest. 


  1. Irani, RA Pettingill, P Kleopa, KA et al. (2012). Morvan syndrome: clinical and serological observations in 29 cases. Ann Neurol 74: 241–255. [PubMed]  

  2. Panzer, J and Dalmau, J. (2011). Movement disorders in paraneoplastic and autoimmune disease. Curr Opin Neurol 24: 346–353. [PubMed]  

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