Category: Choreas (Non-Huntington's Disease)
Objective: Describe a case of hemichorea as the initial symptom of CASPR2 encephalitis.
Background: The clinical spectrum of syndromes associated with CASPR2 is extensive and frequently associated with neuromyotonia and encephalitis. CASPR2-encephalitis is rarely associated with chorea. To our knowledge, only two cases have been reported in the literature
Method: Retrospective analysis of data obtained in medical history and review of literature
Results:
A 78-year-old female patient presented a history of hemichorea for a the past 3-month, progressing with anxiety disorder, paranoid ideas, and generalized chorea. She presented MRI and EEG within normal parameters, MMSE: 19/30, MOCA: 13/30 and CASPR2 antibody serum-positive by indirect immunofluorescence test. PET-CT was normal.
The patient has received solumedrol 1gr/day for five days and subsequent maintenance with meprednisone, with complete remission of symptoms.
Conclusion:
Chorea in CASPR2 syndrome is rare and it has only been described in two previous clinical cases. Immune-mediated disorders should be considered as differential diagnoses in patients with hemichorea; therefore, their study should include a search for CASPR2.
To cite this abstract in AMA style:
A. Ventura, G. Lopez, MA. Piedrabuena, E. Seminara, E. Garino, D. Ballesteros. Hemichorea as an initial symptom of antibodies against contactin- associated protein-like 2 (CASPR2) encephalitis [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/hemichorea-as-an-initial-symptom-of-antibodies-against-contactin-associated-protein-like-2-caspr2-encephalitis/. Accessed May 18, 2025.« Back to MDS Virtual Congress 2021
MDS Abstracts - https://www.mdsabstracts.org/abstract/hemichorea-as-an-initial-symptom-of-antibodies-against-contactin-associated-protein-like-2-caspr2-encephalitis/