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Rhombencephalitis / Brainstem Encephalitis

  • Infection (Burk Jubelt, Section Editor)
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Abstract

Rhombencephalitis (RE) is a syndrome of multiple causes and multiple outcomes. Most authors now use the terms “rhombencephalitis” and “brainstem encephalitis” interchangeably even though anatomically they are slightly different. The etiologic categories of RE include infections, autoimmune diseases, and paraneoplastic syndromes (PNS). Listeria is the most common cause of infectious RE. Listeria RE primary occurs in healthy young adults. It usually occurs as a biphasic time course with a flu-like syndrome followed by brainstem dysfunction; 75% of patients have a cerebrospinal fluid (CSF) pleocytosis, and almost 100% have an abnormal brain MRI scan. Positive CSF and blood cultures are the most specific for diagnosis. Treatment primarily is with ampicillin. Enterovirus 71 is probably the second most common infectious cause of RE; however, 95% of cases have occurred in the Asian-Pacific region and there is no specific treatment. Herpes simplex virus (HSV) is the third most common infectious cause of RE, and about 80% of cases are caused by HSV1 and 20% by HSV2. About 50% only had involvement of the brainstem whereas the other 50% also had supratentorial involvement of the temporal and frontal lobes. Mortality with acyclovir treatment was 22% versus those not on acyclovir 75%. Epstein-Barr virus (EBV) and human herpesvirus 6 (HHV6) have caused a few cases. The most common autoimmune etiology is Behçet disease. Over 90% of those with Behçet RE had abnormal MRI scans and 94% had a CSF pleocytosis. Treatment is with corticosteroids and immunosuppressive agents, but only 25% have complete recovery. Paraneoplastic causes are the third category of RE. Brain MRIs are usually normal; there is usually a CSF pleocytosis but the protein is usually normal. Often anti-neuronal antibodies can be found. Prognosis is poor and treatment is only partially beneficial. Because Listeria and HSV are the most common treatable acute causes of RE, we recommend empiric therapy with ampicillin and acyclovir for all cases after samples have been obtained from CSF and blood for cultures and the polymerase chain reaction (PCR). Antibiotics can be changed based upon MRI, culture results, PCR results, and antibody studies.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Armstrong RW, Fund PC. Brainstem encephalitis (rhombenecephalitis) due to Listeria monocytogenes: case report and literature review. Clin Infect Dis. 1993;16:689–702.

    Article  PubMed  CAS  Google Scholar 

  2. Reynauld L, Graf M, Gentile I, et al. A rare case of brainstem encephalitis by Listeria monocytogenes with isolated mesencephalic localization. Case report and review. Diagn Microbiol Infect Dis. 2007;58:121–3.

    Article  Google Scholar 

  3. Smiatacz T, Kowalik MM, Hlebowicz M. Prolonged dysphagia due to Listeria-rhombencephalitis with brainstem abscess and acute polyradiculoneuritis. J Infect. 2006;52:e165–c167.

    Article  PubMed  Google Scholar 

  4. Crawford JR, Kadom N, Santi MR, et al. Human herpes virus 6 rhombencephalitis in immunocompetent children. J Child Neurol. 2007;22:1260–8.

    Article  PubMed  Google Scholar 

  5. Nichter CA, Pavlakis SG, Shaikh U, et al. Rhombencephalitis caused by West Nile fever virus. Neurology. 2000;55:153.

    PubMed  CAS  Google Scholar 

  6. Medina-Flores R, Germanwala A, Molina JT, et al. October 2003: a 59-year-old woman with sudden onset of diplopia. Brain Pathol. 2004;14:225–6.

    Article  PubMed  Google Scholar 

  7. •• Moragas M, Martinez-Yelamos S, Majos C, et al. Rhombencephalitis. Medicine. 2011;90:256–61. This is a retrospective analysis of RE cases seen over an 18-year period at University Hospital of Bellvitige (Barcelona, Spain).

    Article  PubMed  Google Scholar 

  8. • Huang CC, Liu CC, Chang YC, et al. Neurologic complications in children with enterovirus 71 infection. N Engl J Med. 1999;341:936–42. Excellent clinical report on EV 71 rhombencephalitis.

    Article  PubMed  CAS  Google Scholar 

  9. • Bart R. Listeria and atypical presentations of listeria in the central nervous system. Semin Neurol. 2000;20:361–73. Excellent review of CNS listeriosis.

    Article  Google Scholar 

  10. • Mylonakis E, Hohmann E, Calderwood SB. Central nervous system infection with Listeria monocytogenes: 33 years’ experience at a general hospital and review of 776 episodes from the literature. Medicine. 1998;77:313–36. Excellent clinical report on CNS listeriosis.

    Article  PubMed  CAS  Google Scholar 

  11. Goulet V, Marchetti P. Listeriosis in 225 non-pregnant patients in 1992: clinical aspects and outcome in relation to predisposing conditions. Scand Infect Dis. 1996;28:367–74.

    Article  CAS  Google Scholar 

  12. Eck H. Encephalomyelitis listeriaca apostematosa. Schweiz Med Wochenschr. 1957;87:210–4.

    PubMed  CAS  Google Scholar 

  13. Antal EA, Dietrichs E, Løberg EM, et al. Brainstem encephalitis in listeriosis. Scand J Infect Dis. 2005;37:190–4.

    Article  PubMed  Google Scholar 

  14. Uldry PA, Kuntzer T, Bogousslavsky J, et al. Early symptoms and outcome of Listeria monocytogenes rhombencephalitis: 14 adult cases. J Neurol. 1993;240:235–42.

    Article  PubMed  CAS  Google Scholar 

  15. Alstadhaug KB, Antel EA, Nielsen EW, et al. Listeria rhombencephalitis—a case report. Eur J Neurol. 2006;13:93–102.

    Article  PubMed  CAS  Google Scholar 

  16. • Kayaaslan BU, Akinci E, Bilen S, et al. Listerial rhombencephalitis in an immunocompetent young adult. Intl J Infect Dis. 2009;13:e65–7. This is only a single case report but is an excellent discussion.

    Article  PubMed  Google Scholar 

  17. Popescu GA, Saquepee M, Poisson D, Prazuck T. Treatment difficulties of a listerial rhombencephalitis in an adult patient allergic to penicillins. J Clin Pathol. 2004;57:665–6.

    Article  PubMed  CAS  Google Scholar 

  18. Morosi S, Francisci D, Baldelli F. A case of rhombencephalitis caused by Listeria monocytogenes successfully treated with linezolid. J Infect. 2006;52:e73–5.

    Article  PubMed  CAS  Google Scholar 

  19. Bianchi G, Appollonio I, Piolti R, et al. Listeria rhombencephalitis: report of two cases with early diagnosis and favourable outcome. Clin Neurol Neurosurg. 1995;97:344–8.

    Article  PubMed  CAS  Google Scholar 

  20. Chumakov M, Voroshilova M, Shindarov L. Enterovirus 71 isolated from cases of epidemic poliomyelitis-like disease in Bulgaria. Arch Virol. 1979;60:329–40.

    Article  PubMed  CAS  Google Scholar 

  21. • Ooi MH, Wong SC, Lewthwaite P, et al. Clinical features, diagnosis, and management of enterovirus 71. Lancet Neurol. 2010;9:1097–105. This is an excellent review of EV 71 outbreaks, clinical disease, virological diagnosis, and treatment.

    Article  PubMed  Google Scholar 

  22. Vallet S, Legrand-Quillien M-C, Dailland T, et al. Fatal case of enterovirus 71 infection, France, 2007. Emerg Infect Dis. 2009;15:1837–40.

    PubMed  Google Scholar 

  23. Shen W-C, Chiu H-H, Chow K-C, et al. MR imaging findings of enteroviral encephalomyelitis: an outbreak in Taiwan. Am J Neuroradiol. 1999;20:1889–95.

    PubMed  CAS  Google Scholar 

  24. •• Livorsi D, Anderson E, Qureshi S, et al. Brainstem encephalitis: an unusual presentation of herpes simplex virus infection. J Neurol. 2010;257:1432–7. This is an excellent review of HSV rhombencephalitis/brainstem encephalitis.

    Article  PubMed  Google Scholar 

  25. International Study Group for Behcet’s disease. Criteria for diagnosis of Behcet’s disease. Lancet. 1990;335:1078–80.

    Google Scholar 

  26. Lehner T, Lavery R, Mith R, et al. Association between the 65-kolodalton heat shock protein, streptococcus sanguis and the corresponding auto antibodies in Behcet’s syndrome. Infect Immun. 1991;59:1434–41.

    PubMed  CAS  Google Scholar 

  27. Ohno S, Ohguchi M, Hirose S, et al. Close association of HLS-BW51 with Behcet’s disease. Arch Ophthalmol. 1982;100:1455–8.

    PubMed  CAS  Google Scholar 

  28. Pallis CA, Fudge BJ. The neurological complications of Behcet’s syndrome (review). Arch Neurol Psychiatr. 1956;75:1–14.

    CAS  Google Scholar 

  29. Wadia N, Williams E. Behcet’s syndrome with neurological complications. Brain. 1957;80:59–71.

    Article  PubMed  CAS  Google Scholar 

  30. Kidd D, Steuel A, Denman AM, et al. Neurological complications in Behcet’s syndrome. Brain. 1999;122:2183–94.

    Article  PubMed  Google Scholar 

  31. • Metreau-Vastel J, Mikaeloff Y, Tardieu M, et al. Neurological involvement in pediatric Behcet’s disease. Neuropediatrics. 2010;41:228–34. This is a recent review with up-to-date references.

    Article  PubMed  CAS  Google Scholar 

  32. Akman-Demir G, Serdaroglu P, Tasci B, the Neuro-Behçet Study Group. Clinical patterns of neurological involvement in Behcet’s disease: evaluation of 200 patients. Brain. 1999;122:2171–81.

    Article  PubMed  Google Scholar 

  33. Graus F, Delattre JY, Antoine JC, et al. Recommended diagnostic criteria for paraneoplastic syndromes. JNNP. 2004;75:1135–40.

    CAS  Google Scholar 

  34. •• Dalmau J, Rosenfeld MR. Paraneoplastic syndromes of the CNS. Lancet Neurol. 2008;7:327–40. This is an excellent general review of CNS paraneoplastic syndromes.

    Article  PubMed  Google Scholar 

  35. Rojas I, Grause F, Keime-Guibert F, et al. Long-term clinical outcome of paraneoplastic cerebellar degeneration and anti-Yo antibodies. Neurology. 2000;55:713–5.

    PubMed  CAS  Google Scholar 

  36. Bernal F, Shams’ili S, Rojas I, et al. Anti-Tr antibodies as markers of paraneoplastic cerebellar degeneration and Hodgkin’s disease. Neurology. 2003;60:230–4.

    PubMed  CAS  Google Scholar 

  37. Pittock SJ, Lucchinetti C, Parisi JE, et al. Amphiphysis auto-immunity: paraneoplastic accompaniments. Ann Neurol. 2005;58:96–107.

    Article  PubMed  Google Scholar 

  38. Coppens T, Van den Bergh P, Duprez TJ, et al. Paraneoplastic rhombencephalitis and brachial plexopathy in two cases of amphiphysin auto-immunity. Eur Neurol. 2006;55:80–3.

    Article  PubMed  CAS  Google Scholar 

  39. De Andres C, Esquivel A, De Villoria JG, et al. Unusual magnetic resonance imaging and cerebrospinal fluid findings in paraneoplastic cerebellar degeneration: a sequential study. JNNP. 2006;77:562–3.

    Google Scholar 

  40. •• Saiz A, Bruna J, Stourac P, et al. Anti-Hu-associated brainstem encephalitis. JNNP. 2009;80:404–7. This is an excellent review of 22 cases of brainstem encephalitis.

    CAS  Google Scholar 

  41. Rojas-Marcos I, Rousseau A, Keime-Guibert F, et al. Spectrum of paraneoplastic neurologic disorders in women with breast and gynecologic cancer. Medicine. 2003;82:216–23.

    PubMed  Google Scholar 

  42. • Vigliani MC, Novero D, Cerrato P, et al. Double step paraneoplastic brainstem encephalitis: a clinicopathological study. J Neurol Neurosurg Psychiatr. 2009;80:693–5. This is an interesting and atypical case report.

    Article  PubMed  Google Scholar 

  43. Bodian D. Poliomyelitis: pathologic anatomy. In: Poliomyelitis: papers and discussions presented at the first International Poliomyelitis Conference. Philadelphia, Lippincott; 1949. p. 62–84.

  44. Goto K, Sanefuji M, Kusuhara K, et al. Rhombencephalitis and coxsackievirus A16. Emerg Infect Dis. 2009;15:1689–91.

    PubMed  Google Scholar 

  45. Kuntzer T, Bogousslavsky J, Regli F. Encéphalite “bénigne” du tronc cerebral. Rev Neurol (Paris). 1987;143:737–45.

    CAS  Google Scholar 

  46. Johnson RT, Burke DS, Elwell M, et al. Japanese encephalitis: immunocytochemical studies of viral antigen and inflammatory cells in fatal cases. Ann Neurol. 1985;18:567–73.

    Article  PubMed  CAS  Google Scholar 

  47. Feit H, Luby JP, Mollenrich S. Brainstem encephalitis with serological evidence of St. Louis encephalitis [letter]. J Neurol Neurosurg Psychiatr. 1981;44:368.

    Article  PubMed  CAS  Google Scholar 

  48. Burton JM, Kern RZ, Holliday W, et al. Neurologic manifestations of West Nile virus infection. Can J Neurol Sci. 2004;31:183–93.

    Google Scholar 

  49. Davis LE, DeBiasi R, Goade DE, et al. West Nile virus neuroinvasive disease. Ann Neurol. 2006;60:286–300.

    Article  PubMed  Google Scholar 

  50. Shian WJ, Chi CS. Fatal brainstem encephalitis caused by Epstein-Barr virus. Pediatr Radiol. 1994;24:596–7.

    Article  PubMed  CAS  Google Scholar 

  51. Angelina L, Bugiani M, Zibordi F, et al. Brainstem encephalitis resulting from Epstein-Barr virus mimicking an infiltrating tumor in a child. Pediatr Radiol. 2000;22:130–2.

    Google Scholar 

  52. Duong M, Piroth L, Chavanet P, Comvernoux A, et al. A case of rhombencephalitis with isolation of cytomegalovirus and mycobacterium avium complex in a woman with AIDS. AIDS. 1994;8:1356–7.

    Article  PubMed  CAS  Google Scholar 

  53. Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpes virus infections of the nervous system. Brain Pathol. 2001;11:440–51.

    Article  PubMed  CAS  Google Scholar 

  54. Kocen RS, Parsons M. Neurological complications of tuberculosis: some unusual manifestations. Q J Med. 1970;39:17–30.

    PubMed  CAS  Google Scholar 

  55. Farrell VJ. Brainstem tuberculoma in adult patients: diagnosis and treatment. Surg Neurol. 1990;34:383–9.

    Article  PubMed  CAS  Google Scholar 

  56. Al Deeb SM, Yaqub BA, Sharif HS, Phadke JG. Neurobrucellosis: clinical characteristics, diagnosis and outcome. Neurology. 1989;39:498–501.

    PubMed  Google Scholar 

  57. Kuntzer T, Bogousslavsky J, Miklossy J, et al. Borrelia rhombencephalomyelopathy. Arch Neurol. 1991;48:832–6.

    PubMed  CAS  Google Scholar 

  58. Wali GM. Bickerstaff’s brainstem encephalitis associated with typhoid fever. Postgrad Med J. 1991;67:1011–2.

    Article  PubMed  CAS  Google Scholar 

  59. Platzeck C, Foerster EC, Schneider MC. Encephalitis in Legionella bozemanii pneumonia. Dtsch Med Wochenschr. 1990;115:1956–9.

    Article  PubMed  CAS  Google Scholar 

  60. Hely MA, Williamson PM, Terenty TR. Neurological complications of mycoplasma pneumoniae infection. Clin Exp Neurol. 1984;20:153–60.

    PubMed  CAS  Google Scholar 

  61. Wasenko JJ, Park BJ, Jubelt B, et al. Magnetic resonance imaging of mesenrhombencephalitis. J Clin Image. 2002;26:237–42.

    Article  Google Scholar 

  62. Lanczik O, Lecei O, Schwarz S, Hennerici M. Mycoplasma pneumoniae infection as a treatable cause of brainstem encephalitis. Arch Neurol. 2003;60:1813.

    Article  PubMed  Google Scholar 

  63. Roine RO, Hillbom M, Valle M, et al. Fatal encephalitis caused by a bat-borne rabies-related virus: clinical findings. Brain. 1988;111:1505–16.

    Article  PubMed  Google Scholar 

  64. Bastian FO, Wende RD, Singer DB, Zeller RS. Eastern equine encephalomyelitis: histopathologic and ultrastructural changes with isolation of the virus in a human case. Am J Clin Pathol. 1975;64:10–3.

    PubMed  CAS  Google Scholar 

  65. Zagardo MT, Shanholtz CB, Zoarski GH, Rothman MI. Rhombencephalitis caused by adenovirus: MR imaging appearance. Am J Neuroradiol. 1998;19:1901–3.

    PubMed  CAS  Google Scholar 

  66. Protheroe SM, Mellor DH. Imaging in influenza A encephalitis. Arch Dis Child. 1991;66:702–5.

    Article  PubMed  CAS  Google Scholar 

  67. Woods II ML, Currie BJ, Howard DM, et al. Neurological melioidosis: seven cases from the Northern Territory of Australia. Clin Infect Dis. 1992;15:163–9.

    Article  PubMed  Google Scholar 

  68. Barrios N, Tabbi CK, Rotstein C, et al. Brainstem invasion by aspergillus fumigatus in a child with leukemia. New York State J Med. 1988;88:656–8.

    CAS  Google Scholar 

  69. Macdonell RA, Donnan RA, Kalnins RM, et al. Otocerebral mucormycosis—a case report. Clin Exp Neurol. 1987;23:225–32.

    PubMed  CAS  Google Scholar 

  70. Bertoldi RV, Sperling MR. Nocardia brainstem abscess: diagnosis and response to medical therapy. Bull Clin Neurosci. 1984;49:99–104.

    PubMed  CAS  Google Scholar 

  71. Sotelo J, Guerrero V, Rubio F. Neurocysticercosis: a new classification based on active and inactive forms: a study of 753 cases. Arch Intern Med. 1985;145:442–5.

    Article  PubMed  CAS  Google Scholar 

  72. Kure K, Harris C, Morin LS, Dickson DW. Solitary midbrain toxoplasmosis and olivary hypertrophy in a patient with acquired immunodeficiency syndrome. Clin Neuropathol. 198;8:35–40.

  73. Johnson RT, Richardson EP. The neurological manifestations of systemic lupus erythematosus: a clinical-pathological study of 24 cases and review of the literature. Medicine (Baltimore). 1968;47:337–9.

    Article  CAS  Google Scholar 

  74. Wasserfallen J-B, Schaller MD. Unusual rhombencephalitis in relapsing polychondritis. Ann Rheum Dis. 1992;51:1184.

    Article  PubMed  CAS  Google Scholar 

  75. Deves D, Bombois S, Ferriby D, et al. Acute rhombencephalitis with fever and bilateral hearing loss secondary to Gougerot-Sjogren’s syndrome. Rev Neuro (Paris). 2002;158:211–4.

    Google Scholar 

  76. Oksanen V. Neurosarcoidosis: clinical presentations and course in 50 patients. Acta Neurol Scand. 1986;73:283–90.

    Article  PubMed  CAS  Google Scholar 

  77. Hashimoto T, Takizawa H, Yukimura K, Ohta K. Vogt-Koyanagi-Harada disease associated with brainstem encephalitis. J Clin Neurosci. 2009;16:593–5.

    Article  PubMed  Google Scholar 

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Disclosure

B. Jubelt is an upaid advisory board member for the National Multiple Sclerosis Society (New York Upstate Chapter) and for the Multiple Sclerosis Resources of Central New York. He is a consultant for EMD Serono, Novartis, Biogen, and Teva Neuroscience. He has received grants paid to his institution from Serono, Pfizer, Biogen, Genzyme, EMD Serono, Novartis, and Sanofi-Aventis, and he has received honoraria from the National Multiple Sclerosis Society and Multiple Sclerosis Resources of Central New York. He has received payment for development of continuing medical education presentations from TCL Institutes and Prime Education Inc., and has received payment for service on speakers’ bureaus from TEVA Neuroscience, EMD Serono, Biogen, and Bayer Health Care. His travel expenses have also been reimbursed for his work as a consultant, speaking engagements, CME talks, and his work on speakers’ bureaus. C. Mihai: none; T. Li: none; P. Veerapaneni; none.

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  1. Terrence M. Li

    Present address: Formally Department of Neurology, SUNY Upstate Medical University, Advocate Medical Group, 825 Golf Road, Arlington Heights, IL, 60050, USA

  2. Padma Veerapaneni

    Present address: Formally Department of Neurology, SUNY Upstate Medical University, KIMS Hospital, 1-8-311 Minester’s Road, Secundrabad, 500003, Andhra Pradesh, India

Authors and Affiliations

  1. Departments of Neurology, Microbiology/Immunology and Neuroscience, SUNY Upstate Medical University, 750 E. Adams St, Syracuse, NY, 13210, USA

    Burk Jubelt

  2. Syracuse Veterans Affairs Medical Center, 800 Irving Ave, Syracuse, NY, 13210, USA

    Burk Jubelt

  3. Syracuse Veterans Affairs Medical Center, 800 Irving Ave, Syracuse, NY, 13210, USA

    Cornelia Mihai

  4. The Department of Neurology, SUNY Upstate Medical University, 750 E. Adams St, Syracuse, NY, 13210, USA

    Cornelia Mihai

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Jubelt, B., Mihai, C., Li, T.M. et al. Rhombencephalitis / Brainstem Encephalitis. Curr Neurol Neurosci Rep 11, 543–552 (2011). https://doi.org/10.1007/s11910-011-0228-5

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