Advertisement

A case of fulminant Epstein-Barr virus encephalitis in an immune-competent adult

  • Marvyn T. KoningEmail author
  • Tessa Brik
  • Rogier Hagenbeek
  • Ido van den Wijngaard
Case Report

Abstract

A 21-year-old female presented with headache, nausea and vomiting, dysarthria, difficulty finding words, vertigo, episodical diplopia and an abnormal gait since 2 days. Additionally, we found marked ataxia and disturbed liver chemistry whilst her infection parameters were low. Her head CT scan was unremarkable, but her MRI scan showed leptomeningeal enhancement along the cerebellar folia. A lumbar punction revealed mononuclear leucocytosis and increased protein in her cerebrospinal fluid. She was admitted on a working diagnosis of herpes simplex encephalitis. Shortly after admission, she had a generalised seizure. She was tested for a wide range of viruses, bacteria and auto-immune antibodies and treated empirically with aciclovir, ceftriaxone, doxycycline and intravenous immunoglobulins. All tests continued to come back negative until the fifth day of admission, when repeat Epstein-Barr virus (EBV) serology showed evidence of an acute EBV infection, even though negative tests were acquired at admission. EBV encephalitis is a rare complication of EBV infection that is usually restricted to children and immune-compromised individuals. This is only the fifth case describing EBV encephalitis in an immune-competent adult, presenting with unique clinical features including a lack of fever and leptomeningeal enhancement on MRI investigation. Most interestingly, she tested negative for EBV until a few days after admission, underlining the need for repeated investigations in suspected virological encepahlitis. Even though our patient did not receive the often recommended glucocorticosteroids, she made a good neurological recovery.

Keywords

EBV Encephalitis Cerebellitis Epilepsy 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Written informed consent for publication was obtained from the patient prior to submission.

References

  1. Abolhassani H, Edwards ES, Ikinciogullari A, Jing H, Borte S, Buggert M, Du L, Matsuda-Lennikov M, Romano R, Caridha R, Bade S, Zhang Y, Frederiksen J, Fang M, Bal SK, Haskologlu S, Dogu F, Tacyildiz N, Matthews HF, McElwee JJ, Gostick E, Price DA, Palendira U, Aghamohammadi A, Boisson B, Rezaei N, Karlsson AC, Lenardo MJ, Casanova JL, Hammarstrom L, Tangye SG, Su HC, Pan-Hammarstrom Q (2017) Combined immunodeficiency and Epstein-Barr virus-induced B cell malignancy in humans with inherited CD70 deficiency. J Exp Med 214:91–106CrossRefGoogle Scholar
  2. Ascencao BB, Goncalves AC, Luis N, Sa J, Brito AP, Pocas JM (2016) Epstein-Barr virus hemorrhagic meningoencephalitis: case report and review of the literature. J Neuro-Oncol 22:695–698Google Scholar
  3. Barberi W, Perrone S, Iori AP, Torelli GF, Testi AM, Moleti ML, Ceglie T, Papoff P, Caresta E, Antonelli M, Gianno F, Melone A, Badiali M, Giangaspero F, Foa R, Gentile G (2015) Proven Epstein-Barr encephalitis with negative EBV-DNA load in cerebrospinal fluid after allogeneic hematopoietic stem cell transplantation in a child with acute lymphoblastic leukemia. Pediatr Transplant 19:E19–E24CrossRefGoogle Scholar
  4. Di Carlo P, Trizzino M, Titone L, Capra G, Colletti P, Mazzola G, Pistoia D, Sarno C (2011) Unusual MRI findings in an immunocompetent patient with EBV encephalitis: a case report. BMC Med Imaging 11:6CrossRefGoogle Scholar
  5. Doja A, Bitnun A, Jones EL, Richardson S, Tellier R, Petric M, Heurter H, Macgregor D (2006) Pediatric Epstein-Barr virus-associated encephalitis: 10-year review. J Child Neurol 21:385–391CrossRefGoogle Scholar
  6. Hussain RS, Hussain NA (2013). Ataxia and encephalitis in a young adult with EBV mononucleosis: a case report. Case Rep Neurol Med 2013: 516325Google Scholar
  7. Lau JSY, Low ZM, Abbott I, Shochet L, Kanellis J, Kitching AR, Korman TM (2017) Epstein-Barr virus encephalitis in solid organ transplantation. New Microbiol 40:212–217PubMedGoogle Scholar
  8. Polilli E, Sozio F, Mazzotta E, Consorte A, Di Masi F, Agostinone A, Tontodonati M, Cosentino L, Parruti G (2010) Rapidly progressive and fatal EBV-related encephalitis in a patient with advanced HIV-1 infection at presentation: a case report and review of the literature. New Microbiol 33:275–280PubMedGoogle Scholar
  9. Rafailidis PI, Mavros MN, Kapaskelis A, Falagas ME (2010) Antiviral treatment for severe EBV infections in apparently immunocompetent patients. J Clin Virol 49:151–157CrossRefGoogle Scholar
  10. Schellinger PD, Sommer C, Leithauser F, Schwab S, Storch-Hagenlocher B, Hacke W, Kiessling M (1999) Epstein-Barr virus meningoencephalitis with a lymphoma-like response in an immunocompetent host. Ann Neurol 45:659–662CrossRefGoogle Scholar

Copyright information

© Journal of NeuroVirology, Inc. 2019

Authors and Affiliations

  1. 1.Department of NeurologyHaaglanden Medical CenterThe HagueThe Netherlands
  2. 2.Department of HematologyLeiden University Medical CenterLeidenThe Netherlands
  3. 3.Department of RadiologyHaaglanden Medical CenterThe HagueThe Netherlands

Personalised recommendations