Skip to main content

Advertisement

SpringerLink
Log in

Brainstem encephalitis: an unusual presentation of herpes simplex virus infection

  • Review
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Herpes simplex virus (HSV) encephalitis has a predilection for the temporal and frontal lobes but occasionally affects the brainstem. We describe a patient who developed HSV brainstem encephalitis that progressed to quadriplegia. Using MEDLINE, we conducted a comprehensive review of other published cases of HSV brainstem encephalitis. Twenty-four published cases of HSV brainstem encephalitis met our inclusion criteria. The mean age was 41.4 years (range 18–71). HSV-1 was the etiologic agent in 79% of reported HSV brainstem encephalitis cases, and HSV-2 accounted for 21% of cases. Infection was limited to the brainstem in 29% of cases and multi-focal, including the brainstem, in 71%. Common manifestations of HSV brainstem encephalitis included neuro-ophthalmologic findings (81%), cranial nerve deficits (69%), and fever (69%). Quadriplegia, as occurred in our patient, was an unusual finding (19%). The mortality rate of HSV brainstem encephalitis was 41%. Intravenous acyclovir showed a beneficial effect on mortality (75% vs. 22%, p = 0.06). HSV brainstem encephalitis is a distinct type of HSV encephalitis. With the increasing use of HSV-PCR, more cases of HSV brainstem encephalitis may be identified. A greater recognition of this syndrome will help better define its optimal treatment and prognosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Hjalmarsson A, Blomqvist P, Skoldenberg B (2007) Herpes simplex encephalitis in Sweden, 1990–2001: incidence, morbidity, and mortality. Clin Infect Dis 45:875–880

    Article  PubMed  Google Scholar 

  2. Whitley RJ, Gnann JW (2002) Viral encephalitis: familiar infections and emerging pathogens. Lancet 359:507–513

    Article  PubMed  Google Scholar 

  3. Domingues RB, Tsanaclis AM, Pannuti CS, Mayo MS, Lakeman FD (1997) Evaluation of the range of clinical presentations of herpes simplex encephalitis by using polymerase chain reaction assay of cerebrospinal fluid samples. Clin Infect Dis 25:86–91

    Article  CAS  PubMed  Google Scholar 

  4. Kessler HH, Mühlbauer G, Rinner B, Stelzl E, Berger A, Dörr HW, Santner B, Marth E, Rabenau H (2000) Detection of herpes simplex virus DNA by real-time PCR. J Clin Microbiol 38:2638–2642

    CAS  PubMed  Google Scholar 

  5. Chu K, Kang DW, Lee JJ, Yoon BW (2002) Atypical brainstem encephalitis caused by HSV-2. Arch Neurol 59:460–463

    Article  PubMed  Google Scholar 

  6. Domingues RB, Fink MC, Tsanaclis AM, de Castro CC, Cerri GG, Mayo MS, Lakeman FD (1998) Diagnosis of herpes simplex encephalitis by magnetic resonance imaging and polymerase chain reaction assay of cerebrospinal fluid. J Neurol Sci 157:148–153

    Article  CAS  PubMed  Google Scholar 

  7. Duarte J, Argente J, Gutierrez P, Catalan MJ, Cabello A, Clavería LE (1994) Herpes simplex brainstem encephalitis with a relapsing course. J Neurol 241:401–403

    Article  CAS  PubMed  Google Scholar 

  8. Hamilton RL, Achim C, Grafe MR, Fremont JC, Miners D, Wiley CA (1995) Herpes simplex virus brainstem encephalitis in an AIDS patient. Clinical Neuropathol 14:45–50

    CAS  Google Scholar 

  9. Hirst LW, Clark AW, Wolinsky JS, Zee DS, Kaizer H, Miller NR, Tutschka PJ, Santos GW (1983) Downbeat nystagmus: a case report of herpetic brain stem encephalitis. J Clin Neuroophthalmol 3:245–249

    CAS  PubMed  Google Scholar 

  10. Jereb M, Lainscak M, Marin J, Popovic M (2005) Herpes simplex virus infection limited to the brainstem. Wien Klin Wochenshr 117:495–499

    Article  Google Scholar 

  11. Jha S, Jose M, Kumar V (2003) Herpes simplex encephalitis: some interesting presentations. Neurol India 51:399–400

    CAS  PubMed  Google Scholar 

  12. Lack B, Atack E (1988) Disseminated hemorrhagic leukoencephalomyelitis with localized herpes simplex brain stem infection. Acta Neuropathol 75:354–361

    Article  Google Scholar 

  13. Latronico N, Candiani A (1987) Brainstem herpes viral encephalitis. Lancet 2:690–691

    Article  CAS  PubMed  Google Scholar 

  14. Martin JR, Holt RK, Webster H (1988) Herpes-simplex-related antigen in human demyelinative disease and encephalitis. Acta Neuropathol 76:325–337

    Article  CAS  PubMed  Google Scholar 

  15. Miura S, Kurita T, Noda K, Ayabe M, Aizawa H, Taniwaki T (2009) Symmetrical brainstem encephalitis caused by herpes simplex virus. J Clin Neurosci 16:589–590

    Article  PubMed  Google Scholar 

  16. Moulignier A, Baudrimont M, Martin-Negrier ML, Mikol J, Lapresle C, Dupont B (1996) Fatal brain stem encephalitis due to herpes simplex virus type 1 in AIDS. J Neurol 243:491–493

    Article  CAS  PubMed  Google Scholar 

  17. Nicoll JA, Love S, Kinrade E (1993) Distribution of herpes simplex virus DNA in the brains of human long-term survivors of encephalitis. Neurosci Lett 157:215–218

    Article  CAS  PubMed  Google Scholar 

  18. Osih RB, Brazie M, Kanno M (2007) Multifocal herpes simplex virus type 2 encephalitis in a patient with AIDS. AIDS Read 17:67–70

    PubMed  Google Scholar 

  19. Roman-Campos G, Toro G (1980) Herpetic brainstem encephalitis. Neurology 30:981–985

    CAS  PubMed  Google Scholar 

  20. Rose JW, Stroop WG, Matsuo F, Henkel J (1992) Atypical herpes simplex encephalitis: clinical, virologic, and neuropathologic evaluation. Neurology 42:1809–1812

    CAS  PubMed  Google Scholar 

  21. Sakakibara R, Hattori T, Fukutake T, Mori M, Yamanishi T, Yasuda K (1998) Micturitional disturbance in herpetic brainstem encephalitis; contribution of the pontine micturition centre. J Neurol Neurosurg Psychiatry 64:269–272

    Article  CAS  PubMed  Google Scholar 

  22. Shoji H, Koga M, Kusuhara T, Kaji M, Ayabe M, Hino H, Hondo R (1994) Differentiation of herpes simplex virus 1 and 2 in cerebrospinal fluid of patients with HSV encephalitis and meningitis by stringent hybridization of PCR-amplified DNA. J Neurol 241:526–530

    Article  CAS  PubMed  Google Scholar 

  23. Tang JW, Coward LJ, Davies NW, Geretti AM, Howard RS, Hirsch NP, Ward KN (2003) Brain stem encephalitis caused by primary herpes simplex 2 infection in a young woman. J Neurol Neurosurg Psychiatry 74:1323–1325

    Article  CAS  PubMed  Google Scholar 

  24. Kaji M, Kusuhara T, Ayabe M, Hino H, Shoji H, Nagao T (1996) Survey of herpes simplex virus infections of the central nervous system, including acute disseminated encephalomyelitis, in the Kyushu and Okinawa regions of Japan. Mult Scler 2:83–87

    CAS  PubMed  Google Scholar 

  25. Dayan AD, Gooddy W, Harrison MJ, Rudge P (1972) Brain stem encephalitis caused by Herpesvirus hominis. Br Med J 4:405–406

    Article  CAS  PubMed  Google Scholar 

  26. Martino GV, Grimaldi LM, Franciotta DM, Piccolo G, Brustia R, Minali L, Melzi d’Eril GV (1990) Isoelectric focusing herpes simplex virus-gB overlay study in brainstem encephalitis. Acta Neurol Scand 82:364–367

    Article  CAS  PubMed  Google Scholar 

  27. Tyler KL, Tedder DG, Yamamoto LJ, Klapper JA, Ashley R, Lichtenstein KA, Levin MJ (1995) Recurrent brainstem encephalitis associated with herpes simplex virus type 1 DNA in cerebrospinal fluid. Neurology 45:2246–2250

    CAS  PubMed  Google Scholar 

  28. Jain S, Maheshwari MC (1984) Herpetic brain-stem encephalitis with features of diencephalic epilepsy. Acta Neurol 6:1–4

    CAS  Google Scholar 

  29. Nakajima H, Furutama D, Kimura F, Shinoda K, Nakagawa T, Shimizu A, Ohsawa N (1995) Herpes simplex virus type 2 infections presenting as brainstem encephalitis and recurrent myelitis. Intern Med 34:839–842

    Article  CAS  PubMed  Google Scholar 

  30. Mertens G, Ieven M, Ursi D, Pattyn SR, Martin JJ, Parizel PM (1993) Detection of herpes simplex virus in the cerebrospinal fluid of patients with encephalitis using the polymerase chain reaction. J Neurol Sci 118:213–216

    Article  CAS  PubMed  Google Scholar 

  31. Matsumara K, Sakuta M (1987) Oculogyric crisis in acute herpetic brainstem encephalitis. J Neurol Neurosurg Psychiatry 50:365–366

    Article  Google Scholar 

  32. Aurelius E, Johansson B, Sköldenberg B, Forsgren M (1993) Encephalitis in immunocompetent patients due to herpes simplex virus type 1 or 2 as determined by specific polymerase chain reaction and antibody assays of cerebrospinal fluid. J Med Virol 39:179–186

    Article  CAS  PubMed  Google Scholar 

  33. McGrath N, Anderson NE, Croxson MC, Powell KF (1997) Herpes simplex encephalitis treated with acyclovir: diagnosis and long-term outcome. J Neurol Neurosurg Psychiatry 63:321–326

    Article  CAS  PubMed  Google Scholar 

  34. Hall W (1993) Infectious lesions of the brainstem. Neurosurg Clin N Am 4:543–551

    CAS  PubMed  Google Scholar 

  35. Aurelius E, Johansson B, Sköldenberg B, Staland A, Forsgren M (1991) Rapid diagnosis of herpes simplex encephalitis by nested polymerase reaction assay of cerebrospinal fluid. Lancet 337:189–192

    Article  CAS  PubMed  Google Scholar 

  36. Lakeman FD, Whitley RJ (1995) Diagnosis of herpes simplex encephalitis. Application of polymerase chain reaction to cerebrospinal fluid from brain-biopsied patients and correlation with disease. National Institute of Allergy and Infectious Disease Collaborative Antiviral Study Group. J Infect Dis 171:857–863

    CAS  PubMed  Google Scholar 

  37. Sköldenberg B, Forsgren M, Alestig K, Bergström T, Burman L, Dahlqvist E, Forkman A, Frydén A, Lövgren K, Norlin K, Norrby R, Olding-Stenkvist I, Stiernnstedt G, Uhnoo I, De Vahl K (1984) Acyclovir versus vidarabine in herpes simplex encephalitis. Randomized multicentre study in consecutive Swedish patients. Lancet 2:707–711

    Article  PubMed  Google Scholar 

  38. Whitley RJ, Alford CA, Hirsch MS, Schooley RT, Luby JP, Aoki FY, Hanley D, Nahmias AJ, Soong SJ (1986) Vidarabine versus acyclovir therapy in herpes simplex encephalitis. N Engl J Med 314:144–149

    Article  CAS  PubMed  Google Scholar 

  39. Raschilas F, Wolff M, Delatour F, Chaffaut C, De Broucker T, Chevret S, Lebon P, Canton P, Rozenberg F (2002) Outcome of and prognostic factors for herpes simplex encephalitis in adult patients: results of a multicenter study. Clin Infect Dis 35:254–260

    Article  PubMed  Google Scholar 

  40. Meyding-Lamade UK, Oberlinner C, Rau PR, Seyfer S, Heiland S, Sellner J, Wildemann BT, Lamadé WR (2003) Experimental herpes simplex virus encephalitis: a combination therapy of acyclovir and glucocorticoids reduces long-term magnetic resonance imaging abnormalities. J Neurovirol 9:118–125

    CAS  PubMed  Google Scholar 

  41. Kamei S, Sekizawa T, Shiota H, Mizutani T, Itoyama Y, Takasu T, Morishima T, Hirayanagi K (2005) Evaluation of combination therapy using aciclovir and corticosteroid in adult patients with herpes simplex virus encephalitis. J Neurol Neurosurg Psychiatry 76:1544–1549

    Article  CAS  PubMed  Google Scholar 

Download references

Conflict of interest statement

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Infectious Disease, Emory University School of Medicine, 49 Jesse Hill Jr Drive, Atlanta, GA, 30303, USA

    Daniel Livorsi & Carlos Franco-Paredes

  2. Department of Neurology, Emory University School of Medicine, WMRB 6000, 1639 Pierce Dr NE, Atlanta, GA, 30322, USA

    Eric Anderson & Sakib Qureshi

  3. Department of Internal Medicine, Emory University School of Medicine, 69 Jesse Hill Jr Drive SE, Atlanta, GA, 30303, USA

    Marion Howard

  4. Pathology and Laboratory Medicine, Grady Memorial Hospital, Emory University School of Medicine, P.O. Box 26248, 80 Jesse Hill Dr SE, Atlanta, GA, 30303, USA

    Yun F. Wang

  5. Division of Infectious Disease, Emory University School of Medicine, TravelWell Clinic, 550 Peachtree Street, MOT 7th Floor, Atlanta, GA, 30308, USA

    Carlos Franco-Paredes

Authors
  1. Daniel Livorsi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Eric Anderson
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sakib Qureshi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Marion Howard
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Yun F. Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Carlos Franco-Paredes
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Daniel Livorsi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Livorsi, D., Anderson, E., Qureshi, S. et al. Brainstem encephalitis: an unusual presentation of herpes simplex virus infection. J Neurol 257, 1432–1437 (2010). https://doi.org/10.1007/s00415-010-5600-x

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-010-5600-x

Keywords

Search

Navigation