Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020 Feb 5;12(1):50-55.
doi: 10.1159/000505232. eCollection 2020 Jan-Apr.

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

Affiliations
Case Reports

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

Ismail Ibrahim Ismail et al. Case Rep Neurol. .

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.

PubMed Disclaimer

Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
MR brain. Axial (a) and coronal (b) T2 showing bilateral hyperintensity involving medial temporal lobes with hippocampal sclerosis and atrophy.
Fig. 2
Fig. 2
CT of the chest showing anterior mediastinal mass (thymoma) measuring 40.7 × 17.2 mm.

References

    1. Dalmau J, Rosenfeld MR. Paraneoplastic syndromes of the CNS. Lancet Neurol. 2008 Apr;7((4)):327–40. - PMC - PubMed
    1. de Beukelaar JW, Sillevis Smitt PA. Managing paraneoplastic neurological disorders. Oncologist. 2006 Mar;11((3)):292–305. - PubMed
    1. Honnorat J, Antoine JC. Paraneoplastic neurological syndromes. Orphanet J Rare Dis. 2007 May;2((1)):22. - PMC - PubMed
    1. de Graaf JH, Tamminga RY, Kamps WA. Paraneoplastic manifestations in children. Eur J Pediatr. 1994 Nov;153((11)):784–91. - PubMed
    1. Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain. 2000 Jul;123((Pt 7)):1481–94. - PubMed

Publication types