Introduction Rare cases of double positive Epstein-Barr virus (EBV) and anti-N-methyl-D-aspartate receptor (anti-NMDAR) antibody causing encephalitis have been described associated with solid organ transplant.^1–4 Opsoclonus-myoclonus syndrome is often a paraneoplastic or parainfectious phenomenon, but has only rarely been described associated with anti-NMDAR and usually with additional neuropsychiatric symptoms or encephalopathy.^5–6 We describe the rare case of a renal transplant patient with opsoclonus-myoclonus syndrome associated with renal cell carcinoma and anti-NMDAR and EBV DNA detected in cerebrospinal fluid (CSF).

Methods This was a personal case of the authors, with documentation and investigations reviewed from patient medical records at Sir Charles Gairdner Hospital.

Results A 55 years old immunocompromised female patient presented with two weeks of increasing ataxia, oscillopsia and tremor, preceded by 3 weeks of headache, nausea and vomiting. This was on a background of three failed renal transplants for IgA focal segmental glomerulosclerosis and recent resection of stage I renal clear cell carcinoma. Examination was consistent with opsoclonus-myoclonus-ataxia syndrome. There were no features on encephalopathy apart from mild emotional lability.. CSF results: lymphocytosis with an elevated protein, positive EBV using polymerase chain reaction and positive anti-NMDAR. Treatment: Plasma exchange, Rituximab, intravenous acyclovir for two weeks followed by oral acyclovir for 3 months. Improvement was marked, although with residual myoclonus on 2 month follow-up.

Conclusions This rare case of paraneoplastic double-positive EBV and anti-NMDAR opsoclonus-myoclonus syndrome in an immunocompromised patient demonstrates the broadening clinical phenotype of anti-NMDAR and highlights the contentious issue of EBV pathogenicity and treatment in an immunocompromised patient.

References

1. Garre, et al. Letter to the editor: EBV-NMDA double positive encephalitis in an immunocompromised patient. Journal of the Neurological Sciences 2019;(396):76–77.

2. Zhao CZ, Erickson J, Clinical Reasoning Dalmau J. Agitation and psychosis in a patient after renal transplantation. Neurology 2012;79:e41–e44.

3. Derksen S, Goraj B, Molenaar J, van der Hoeven JJC. Severe anti NMDA encephalitis and EBV infection. Netherlands J Critic Care 2013;17:19–21.

4. Cohen DA, Lopez-Chiriboga AS, Pittock SJ, Gadoth A, Zekeridou A, Boilson BA, et al. Posttransplant autoimmune encephalitis. Neurol Neuroimmunol Neuroinflam 2018;5:e497.

5. Armangué T, et al. Clinical and immunological features of opsoclonus-myoclonus syndrome in the era of neuronal cell surface antibodies. JAMA Neurology 2016;73(4):417–424.

6. Klaas JP, et al. Adult-onset opsoclonus-myoclonus syndrome. Archives of Neurology 2012;69(12):1598–1607.