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Encephalitis due to human herpesvirus 6 infection

MedGen UID:
363034
Concept ID:
C1955629
Disease or Syndrome
Synonyms: HHV-6 encephalitis; Human Herpesvirus 6 encephalitis; Variant A or HHV-6A; Variant B or HHV-6B
SNOMED CT: Encephalitis caused by human herpesvirus 6 infection (441512004)
 
Monarch Initiative: MONDO:0027029

Definition

HHV-6 encephalitis refers to inflammation of the brain due to an infection with human herpesvirus 6. People who have undergone allogeneic hematopoietic cell transplantation are at an increased risk for developing HHV-6 encephalitis, particularly when umbilical cord blood stem cells are used. People with immune system disorders may also be at an increased risk for developing this infection. Signs and symptoms vary, but often include confusion, anterograde amnesia (difficulty learning new information following the onset of amnesia), short-term memory loss, and seizures.Diagnosis often involves lumbar puncture, virus testing, and MRI. EEG 's may also be recommendedwhen seizures are suspected. HHV-6 encephalitis is treated with an antiviral agent with activity against HHV-6. Long term outlook (chance of full recovery) can vary considerably depending individual patient factors. [from MONDO]

Professional guidelines

PubMed

Costa BKD, Sato DK
J Pediatr (Rio J) 2020 Mar-Apr;96 Suppl 1(Suppl 1):12-19. Epub 2019 Sep 9 doi: 10.1016/j.jped.2019.07.006. PMID: 31513761Free PMC Article
Hill JA
Curr Opin Infect Dis 2019 Dec;32(6):584-590. doi: 10.1097/QCO.0000000000000592. PMID: 31567413Free PMC Article
Ward KN, Hill JA, Hubacek P, de la Camara R, Crocchiolo R, Einsele H, Navarro D, Robin C, Cordonnier C, Ljungman P; 2017 European Conference on Infections in Leukaemia (ECIL)
Haematologica 2019 Nov;104(11):2155-2163. Epub 2019 Aug 29 doi: 10.3324/haematol.2019.223073. PMID: 31467131Free PMC Article

Recent clinical studies

Etiology

Kampouri E, Little JS, Crocchiolo R, Hill JA
Curr Opin Infect Dis 2024 Aug 1;37(4):245-253. Epub 2024 May 6 doi: 10.1097/QCO.0000000000001020. PMID: 38726832
Lareau CA, Yin Y, Maurer K, Sandor KD, Daniel B, Yagnik G, Peña J, Crawford JC, Spanjaart AM, Gutierrez JC, Haradhvala NJ, Riberdy JM, Abay T, Stickels RR, Verboon JM, Liu V, Buquicchio FA, Wang F, Southard J, Song R, Li W, Shrestha A, Parida L, Getz G, Maus MV, Li S, Moore A, Roberts ZJ, Ludwig LS, Talleur AC, Thomas PG, Dehghani H, Pertel T, Kundaje A, Gottschalk S, Roth TL, Kersten MJ, Wu CJ, Majzner RG, Satpathy AT
Nature 2023 Nov;623(7987):608-615. Epub 2023 Nov 8 doi: 10.1038/s41586-023-06704-2. PMID: 37938768Free PMC Article
Kampouri E, Zamora D, Kiem ES, Liu W, Ibrahimi S, Blazevic RL, Lovas EA, Kimball LE, Huang ML, Jerome KR, Ueda Oshima M, Mielcarek M, Zerr DM, Boeckh MJ, Krantz EM, Hill JA
Clin Microbiol Infect 2023 Nov;29(11):1450.e1-1450.e7. Epub 2023 Jul 31 doi: 10.1016/j.cmi.2023.07.026. PMID: 37532126Free PMC Article
Berzero G, Campanini G, Vegezzi E, Paoletti M, Pichiecchio A, Simoncelli AM, Colombo AA, Bernasconi P, Borsani O, Di Matteo A, Rossi V, Foiadelli T, Savasta S, Compagno F, Zecca M, Baldanti F, Marchioni E
Neurol Neuroimmunol Neuroinflamm 2021 Mar;8(2) Epub 2021 Jan 12 doi: 10.1212/NXI.0000000000000942. PMID: 33587722Free PMC Article
Leber AL, Everhart K, Balada-Llasat JM, Cullison J, Daly J, Holt S, Lephart P, Salimnia H, Schreckenberger PC, DesJarlais S, Reed SL, Chapin KC, LeBlanc L, Johnson JK, Soliven NL, Carroll KC, Miller JA, Dien Bard J, Mestas J, Bankowski M, Enomoto T, Hemmert AC, Bourzac KM
J Clin Microbiol 2016 Sep;54(9):2251-61. Epub 2016 Jun 22 doi: 10.1128/JCM.00730-16. PMID: 27335149Free PMC Article

Diagnosis

Berzero G, Campanini G, Vegezzi E, Paoletti M, Pichiecchio A, Simoncelli AM, Colombo AA, Bernasconi P, Borsani O, Di Matteo A, Rossi V, Foiadelli T, Savasta S, Compagno F, Zecca M, Baldanti F, Marchioni E
Neurol Neuroimmunol Neuroinflamm 2021 Mar;8(2) Epub 2021 Jan 12 doi: 10.1212/NXI.0000000000000942. PMID: 33587722Free PMC Article
Costa BKD, Sato DK
J Pediatr (Rio J) 2020 Mar-Apr;96 Suppl 1(Suppl 1):12-19. Epub 2019 Sep 9 doi: 10.1016/j.jped.2019.07.006. PMID: 31513761Free PMC Article
Agut H, Bonnafous P, Gautheret-Dejean A
Microbiol Spectr 2016 Jun;4(3) doi: 10.1128/microbiolspec.DMIH2-0007-2015. PMID: 27337451
Agut H, Bonnafous P, Gautheret-Dejean A
Clin Microbiol Rev 2015 Apr;28(2):313-35. doi: 10.1128/CMR.00122-14. PMID: 25762531Free PMC Article
Jubelt B, Mihai C, Li TM, Veerapaneni P
Curr Neurol Neurosci Rep 2011 Dec;11(6):543-52. doi: 10.1007/s11910-011-0228-5. PMID: 21956758

Therapy

Lareau CA, Yin Y, Maurer K, Sandor KD, Daniel B, Yagnik G, Peña J, Crawford JC, Spanjaart AM, Gutierrez JC, Haradhvala NJ, Riberdy JM, Abay T, Stickels RR, Verboon JM, Liu V, Buquicchio FA, Wang F, Southard J, Song R, Li W, Shrestha A, Parida L, Getz G, Maus MV, Li S, Moore A, Roberts ZJ, Ludwig LS, Talleur AC, Thomas PG, Dehghani H, Pertel T, Kundaje A, Gottschalk S, Roth TL, Kersten MJ, Wu CJ, Majzner RG, Satpathy AT
Nature 2023 Nov;623(7987):608-615. Epub 2023 Nov 8 doi: 10.1038/s41586-023-06704-2. PMID: 37938768Free PMC Article
Ward KN, Hill JA, Hubacek P, de la Camara R, Crocchiolo R, Einsele H, Navarro D, Robin C, Cordonnier C, Ljungman P; 2017 European Conference on Infections in Leukaemia (ECIL)
Haematologica 2019 Nov;104(11):2155-2163. Epub 2019 Aug 29 doi: 10.3324/haematol.2019.223073. PMID: 31467131Free PMC Article
Agut H, Bonnafous P, Gautheret-Dejean A
Microbiol Spectr 2016 Jun;4(3) doi: 10.1128/microbiolspec.DMIH2-0007-2015. PMID: 27337451
Agut H, Bonnafous P, Gautheret-Dejean A
Clin Microbiol Rev 2015 Apr;28(2):313-35. doi: 10.1128/CMR.00122-14. PMID: 25762531Free PMC Article
Caserta MT, Mock DJ, Dewhurst S
Clin Infect Dis 2001 Sep 15;33(6):829-33. Epub 2001 Aug 10 doi: 10.1086/322691. PMID: 11512088

Prognosis

Kampouri E, Krantz EM, Xie H, Ibrahimi SS, Kiem ES, Sekhon MK, Liang EC, Cowan AJ, Portuguese A, Green DJ, Albittar A, Huang JJ, Gauthier J, Pérez-Osorio AC, Jerome KR, Zerr DM, Boeckh MJ, Hill JA
Blood 2024 Aug 1;144(5):490-495. doi: 10.1182/blood.2024024145. PMID: 38635788Free PMC Article
Kampouri E, Zamora D, Kiem ES, Liu W, Ibrahimi S, Blazevic RL, Lovas EA, Kimball LE, Huang ML, Jerome KR, Ueda Oshima M, Mielcarek M, Zerr DM, Boeckh MJ, Krantz EM, Hill JA
Clin Microbiol Infect 2023 Nov;29(11):1450.e1-1450.e7. Epub 2023 Jul 31 doi: 10.1016/j.cmi.2023.07.026. PMID: 37532126Free PMC Article
Ogata M, Fukuda T, Teshima T
Bone Marrow Transplant 2015 Aug;50(8):1030-6. Epub 2015 Apr 27 doi: 10.1038/bmt.2015.76. PMID: 25915811
Jubelt B, Mihai C, Li TM, Veerapaneni P
Curr Neurol Neurosci Rep 2011 Dec;11(6):543-52. doi: 10.1007/s11910-011-0228-5. PMID: 21956758
Ogata M
J Clin Exp Hematop 2009 Nov;49(2):57-67. doi: 10.3960/jslrt.49.57. PMID: 19907110

Clinical prediction guides

Lareau CA, Yin Y, Maurer K, Sandor KD, Daniel B, Yagnik G, Peña J, Crawford JC, Spanjaart AM, Gutierrez JC, Haradhvala NJ, Riberdy JM, Abay T, Stickels RR, Verboon JM, Liu V, Buquicchio FA, Wang F, Southard J, Song R, Li W, Shrestha A, Parida L, Getz G, Maus MV, Li S, Moore A, Roberts ZJ, Ludwig LS, Talleur AC, Thomas PG, Dehghani H, Pertel T, Kundaje A, Gottschalk S, Roth TL, Kersten MJ, Wu CJ, Majzner RG, Satpathy AT
Nature 2023 Nov;623(7987):608-615. Epub 2023 Nov 8 doi: 10.1038/s41586-023-06704-2. PMID: 37938768Free PMC Article
Santpere G, Telford M, Andrés-Benito P, Navarro A, Ferrer I
Biomolecules 2020 Nov 6;10(11) doi: 10.3390/biom10111520. PMID: 33172107Free PMC Article
Phan TL, Lautenschlager I, Razonable RR, Munoz FM
Liver Int 2018 Feb;38(2):210-223. Epub 2017 Jul 29 doi: 10.1111/liv.13506. PMID: 28650593
Leber AL, Everhart K, Balada-Llasat JM, Cullison J, Daly J, Holt S, Lephart P, Salimnia H, Schreckenberger PC, DesJarlais S, Reed SL, Chapin KC, LeBlanc L, Johnson JK, Soliven NL, Carroll KC, Miller JA, Dien Bard J, Mestas J, Bankowski M, Enomoto T, Hemmert AC, Bourzac KM
J Clin Microbiol 2016 Sep;54(9):2251-61. Epub 2016 Jun 22 doi: 10.1128/JCM.00730-16. PMID: 27335149Free PMC Article
Hashimoto K, Yasukawa M, Tohyama M
Curr Opin Allergy Clin Immunol 2003 Aug;3(4):255-60. doi: 10.1097/00130832-200308000-00004. PMID: 12865768

Recent systematic reviews

Ocampo FF, Tipones RN, Jamora RDG
Acta Neurol Taiwan 2021 Mar;30(1):21-26. PMID: 34549397
Victoria NC, Phan TL, Agarwal KA
Biol Blood Marrow Transplant 2020 May;26(5):1034-1039. Epub 2020 Feb 3 doi: 10.1016/j.bbmt.2020.01.023. PMID: 32028025
Scheurer ME, Pritchett JC, Amirian ES, Zemke NR, Lusso P, Ljungman P
Bone Marrow Transplant 2013 Apr;48(4):574-80. Epub 2012 Sep 24 doi: 10.1038/bmt.2012.180. PMID: 23000642

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