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065 EBV and NMDA receptor antibody positive opsoclonus-myoclonus syndrome in an immunocompromised patient with renal clear cell carcinoma: a case report
  1. Catherine Ashton1 and
  2. Janavi Dunuwille2
  1. 1Neurology Department, Fiona Stanley Hospital, Murdoch, WA, Australia
  2. 2Department of neurology, Sir Charles Gairdner Hospital, Nedlands, WA, Australia


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.


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  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.

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