Paraneoplastic Limbic Encephalitis Associated with Anti-CV2/CRMP5 Antibodies Secondary to Thymoma in an Adolescent

Case Rep Neurol. 2020 Feb 5;12(1):50-55. doi: 10.1159/000505232. eCollection 2020 Jan-Apr.

Abstract

Paraneoplastic neurological syndromes (PNS) associated with anti-CV2/CRMP5 antibodies are rare in the literature. Various clinical manifestations can occur including paraneoplastic limbic encephalitis (PLE). Thymoma is one of the rare causes that can be associated with this syndrome. It has not been reported in the literature in children or adolescents to the best of our knowledge. We report a case of PLE in a 19-year-old male patient secondary to thymoma that was diagnosed after 5 years of onset. Anti-CV2/CRMP5 antibodies were positive in the serum and became negative after thymectomy. Diagnosis of PNS should be evoked in cases with atypical neurological manifestation and can be confirmed by the presence of onconeuronal antibodies. We report the first pediatric PLE secondary to thymoma associated with anti-CV2/CRMP5 antibodies.

Keywords: CRMP5; CV2; Limbic encephalitis; Onconeuronal antibodies; Paraneoplastic syndromes; Thymoma.

Publication types

  • Case Reports