U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Infectious encephalitis

MedGen UID:
108917
Concept ID:
C0596773
Disease or Syndrome
Synonyms: Encephalitis, Infectious; Infectious Encephalitis
SNOMED CT: Infective encephalitis (312215006)
 
HPO: HP:0002383
Monarch Initiative: MONDO:0020067

Definition

A disorder of the brain caused by an infectious agent that presents with fever, headache, and an altered level of consciousness. There may also be focal or multifocal neurologic deficits, and focal or generalized seizure activity. [from HPO]

Conditions with this feature

Subacute sclerosing panencephalitis
MedGen UID:
52527
Concept ID:
C0038522
Disease or Syndrome
Subacute sclerosing panencephalitis (SSPE) is a fatal neurodegenerative disease caused by persistent central nervous system infection with the measles virus (summary by Torisu et al., 2004).
X-linked agammaglobulinemia
MedGen UID:
65123
Concept ID:
C0221026
Disease or Syndrome
X-linked agammaglobulinemia (XLA) is characterized by recurrent bacterial infections in affected males in the first two years of life. Recurrent otitis is the most common infection prior to diagnosis. Conjunctivitis, sinopulmonary infections, diarrhea, and skin infections are also frequently seen. Approximately 60% of individuals with XLA are recognized as having immunodeficiency when they develop a severe, life-threatening infection such as pneumonia, empyema, meningitis, sepsis, cellulitis, or septic arthritis. S pneumoniae and H influenzae are the most common organisms found prior to diagnosis and may continue to cause sinusitis and otitis after diagnosis and the initiation of gammaglobulin substitution therapy. Severe, difficult-to-treat enteroviral infections (often manifest as dermatomyositis or chronic meningoencephalitis) can be prevented by this treatment. The prognosis for individuals with XLA has improved markedly in the last 25 years as a result of earlier diagnosis, the development of preparations of gammaglobulin that allow normal concentrations of serum IgG to be achieved, and more liberal use of antibiotics.
X-linked agammaglobulinemia with growth hormone deficiency
MedGen UID:
141630
Concept ID:
C0472813
Disease or Syndrome
IGHD3 is characterized by agammaglobulinemia and markedly reduced numbers of B cells, short stature, delayed bone age, and good response to treatment with growth hormone (summary by Conley et al., 1991). For general phenotypic information and a discussion of genetic heterogeneity of IGHD, see 262400.
Familial hemophagocytic lymphohistiocytosis 2
MedGen UID:
400366
Concept ID:
C1863727
Disease or Syndrome
Familial hemophagocytic lymphohistiocytosis-2 (FHL2) is an autosomal recessive disorder of immune dysregulation with onset in infancy or early childhood. It is characterized clinically by fever, edema, hepatosplenomegaly, and liver dysfunction. Neurologic impairment, seizures, and ataxia are frequent. Laboratory studies show pancytopenia, coagulation abnormalities, hypofibrinogenemia, and hypertriglyceridemia. There is increased production of cytokines, such as gamma-interferon (IFNG; 147570) and TNF-alpha (191160), by hyperactivation and proliferation of T cells and macrophages. Activity of cytotoxic T cells and NK cells is reduced, consistent with a defect in cellular cytotoxicity. Bone marrow, lymph nodes, spleen, and liver show features of hemophagocytosis. Chemotherapy and/or immunosuppressant therapy may result in symptomatic remission, but the disorder is fatal without bone marrow transplantation (summary by Dufourcq-Lagelouse et al., 1999, Stepp et al., 1999, and Molleran Lee et al., 2004). For a general phenotypic description and a discussion of genetic heterogeneity of FHL, see 267700.
MHC class II deficiency
MedGen UID:
444051
Concept ID:
C2931418
Disease or Syndrome
A rare autosomal recessive primary immunodeficiency characterized by absence of HLA class II molecules on the surface of immune cells, leading to severely impaired cellular and humoral immune response to foreign antigens, severe CD4+ T-cell lymphopenia, and hypogammaglobulinemia. The disease clinically manifests with early onset of severe and recurrent infections mainly of the respiratory and gastrointestinal tract, protracted diarrhea with failure to thrive, and autoimmune disease, and is frequently fatal in childhood.
Immunodeficiency 37
MedGen UID:
863632
Concept ID:
C4015195
Disease or Syndrome
Any primary immunodeficiency disease in which the cause of the disease is a mutation in the BCL10 gene.
Familial hemophagocytic lymphohistiocytosis type 1
MedGen UID:
1642840
Concept ID:
C4551514
Disease or Syndrome
Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare primary immunodeficiency characterized by a macrophage activation syndrome with an onset usually occurring within a few months or less common several years after birth.
X-linked lymphoproliferative disease due to SH2D1A deficiency
MedGen UID:
1770239
Concept ID:
C5399825
Disease or Syndrome
X-linked lymphoproliferative disease (XLP) has two recognizable subtypes, XLP1 and XLP2. XLP1 is characterized predominantly by one of three commonly recognized phenotypes: Inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis. Dysgammaglobulinemia. Lymphoproliferative disease (malignant lymphoma). XLP2 is most often characterized by HLH (often associated with EBV), dysgammaglobulinemia, and inflammatory bowel disease. HLH resulting from EBV infection is associated with an unregulated and exaggerated immune response with widespread proliferation of cytotoxic T cells, EBV-infected B cells, and macrophages. Dysgammaglobulinemia is typically hypogammaglobulinemia of one or more immunoglobulin subclasses. The malignant lymphomas are typically B-cell lymphomas, non-Hodgkin type, often extranodal, and in particular involving the intestine.

Professional guidelines

PubMed

Au CC, Hon KL, Leung AKC, Torres AR
Recent Pat Inflamm Allergy Drug Discov 2020;14(2):156-165. doi: 10.2174/1872213X14999201124195724. PMID: 33238854
Fillatre P, Crabol Y, Morand P, Piroth L, Honnorat J, Stahl JP, Lecuit M
Med Mal Infect 2017 May;47(3):236-251. Epub 2017 Mar 15 doi: 10.1016/j.medmal.2017.02.004. PMID: 28314470Free PMC Article
Armangue T, Leypoldt F, Dalmau J
Curr Opin Neurol 2014 Jun;27(3):361-8. doi: 10.1097/WCO.0000000000000087. PMID: 24792345Free PMC Article

Recent clinical studies

Etiology

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
Haston JC, Cope JR
Curr Opin Infect Dis 2023 Jun 1;36(3):186-191. Epub 2023 Apr 10 doi: 10.1097/QCO.0000000000000923. PMID: 37093056Free PMC Article
Sonneville R, de Montmollin E, Contou D, Ferrer R, Gurjar M, Klouche K, Sarton B, Demeret S, Bailly P, da Silva D, Escudier E, Le Guennec L, Chabanne R, Argaud L, Ben Hadj Salem O, Thyrault M, Frerou A, Louis G, De Pascale G, Horn J, Helbok R, Geri G, Bruneel F, Martin-Loeches I, Taccone FS, De Waele JJ, Ruckly S, Staiquly Q, Citerio G, Timsit JF; EURECA Investigator Study Group
Intensive Care Med 2023 May;49(5):517-529. Epub 2023 Apr 6 doi: 10.1007/s00134-023-07032-9. PMID: 37022378
Venkatesan A
Continuum (Minneap Minn) 2021 Aug 1;27(4):855-886. doi: 10.1212/CON.0000000000001006. PMID: 34623096
Granerod J, Ambrose HE, Davies NW, Clewley JP, Walsh AL, Morgan D, Cunningham R, Zuckerman M, Mutton KJ, Solomon T, Ward KN, Lunn MP, Irani SR, Vincent A, Brown DW, Crowcroft NS; UK Health Protection Agency (HPA) Aetiology of Encephalitis Study Group
Lancet Infect Dis 2010 Dec;10(12):835-44. Epub 2010 Oct 15 doi: 10.1016/S1473-3099(10)70222-X. PMID: 20952256

Diagnosis

Venkatesan A
Continuum (Minneap Minn) 2021 Aug 1;27(4):855-886. doi: 10.1212/CON.0000000000001006. PMID: 34623096
Ellul MA, Benjamin L, Singh B, Lant S, Michael BD, Easton A, Kneen R, Defres S, Sejvar J, Solomon T
Lancet Neurol 2020 Sep;19(9):767-783. Epub 2020 Jul 2 doi: 10.1016/S1474-4422(20)30221-0. PMID: 32622375Free PMC Article
Stahl JP, Mailles A
Curr Opin Infect Dis 2019 Jun;32(3):239-243. doi: 10.1097/QCO.0000000000000554. PMID: 30921087
Venkatesan A, Murphy OC
Neurol Clin 2018 Nov;36(4):705-724. Epub 2018 Sep 20 doi: 10.1016/j.ncl.2018.07.001. PMID: 30366550
Dubey D, Pittock SJ, Kelly CR, McKeon A, Lopez-Chiriboga AS, Lennon VA, Gadoth A, Smith CY, Bryant SC, Klein CJ, Aksamit AJ, Toledano M, Boeve BF, Tillema JM, Flanagan EP
Ann Neurol 2018 Jan;83(1):166-177. doi: 10.1002/ana.25131. PMID: 29293273Free PMC Article

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
Zhang L, Zhang L, Li F, Liu W, Tai Z, Yang J, Zhang H, Tuo J, Yu C, Xu Z
Front Immunol 2023;14:1118236. Epub 2023 Jan 20 doi: 10.3389/fimmu.2023.1118236. PMID: 36742325Free PMC Article
Venkatesan A
Curr Opin Infect Dis 2019 Jun;32(3):251-258. doi: 10.1097/QCO.0000000000000546. PMID: 31021956
Stahl JP, Mailles A
Curr Opin Infect Dis 2019 Jun;32(3):239-243. doi: 10.1097/QCO.0000000000000554. PMID: 30921087
Fillatre P, Crabol Y, Morand P, Piroth L, Honnorat J, Stahl JP, Lecuit M
Med Mal Infect 2017 May;47(3):236-251. Epub 2017 Mar 15 doi: 10.1016/j.medmal.2017.02.004. PMID: 28314470Free PMC Article

Prognosis

Fillatre P, Mailles A, Stahl JP, Tattevin P; Scientific Committee and Investigators Group
J Crit Care 2023 Oct;77:154300. Epub 2023 May 17 doi: 10.1016/j.jcrc.2023.154300. PMID: 37207520
Zhang L, Zhang L, Li F, Liu W, Tai Z, Yang J, Zhang H, Tuo J, Yu C, Xu Z
Front Immunol 2023;14:1118236. Epub 2023 Jan 20 doi: 10.3389/fimmu.2023.1118236. PMID: 36742325Free PMC Article
Petitgas P, Tattevin P, Mailles A, Fillâtre P, Stahl JP; ENCEIF scientific committee, investigators group
Infection 2023 Aug;51(4):859-867. Epub 2022 Sep 24 doi: 10.1007/s15010-022-01927-3. PMID: 36152225
Dubey D, Pittock SJ, Kelly CR, McKeon A, Lopez-Chiriboga AS, Lennon VA, Gadoth A, Smith CY, Bryant SC, Klein CJ, Aksamit AJ, Toledano M, Boeve BF, Tillema JM, Flanagan EP
Ann Neurol 2018 Jan;83(1):166-177. doi: 10.1002/ana.25131. PMID: 29293273Free PMC Article
Boucher A, Herrmann JL, Morand P, Buzelé R, Crabol Y, Stahl JP, Mailles A
Med Mal Infect 2017 May;47(3):221-235. Epub 2017 Mar 22 doi: 10.1016/j.medmal.2017.02.003. PMID: 28341533

Clinical prediction guides

Finke C
Neurol Neuroimmunol Neuroinflamm 2024 Jan;11(1):e200189. Epub 2023 Dec 7 doi: 10.1212/NXI.0000000000200189. PMID: 38086067Free PMC Article
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
Brisca G, Marini C, Buratti S, Mariani M, Tortora D, Morana G, Pirlo D, Romanengo M, Cannizzaro G, Cordani R, Canzoneri F, Calevo MG, Nobili L, Franciotta D, Castagnola E, Moscatelli A, Mancardi MM
J Neurol 2023 Oct;270(10):5034-5047. Epub 2023 Jul 3 doi: 10.1007/s00415-023-11847-3. PMID: 37400659
Fillatre P, Mailles A, Stahl JP, Tattevin P; Scientific Committee and Investigators Group
J Crit Care 2023 Oct;77:154300. Epub 2023 May 17 doi: 10.1016/j.jcrc.2023.154300. PMID: 37207520
Sonneville R, de Montmollin E, Contou D, Ferrer R, Gurjar M, Klouche K, Sarton B, Demeret S, Bailly P, da Silva D, Escudier E, Le Guennec L, Chabanne R, Argaud L, Ben Hadj Salem O, Thyrault M, Frerou A, Louis G, De Pascale G, Horn J, Helbok R, Geri G, Bruneel F, Martin-Loeches I, Taccone FS, De Waele JJ, Ruckly S, Staiquly Q, Citerio G, Timsit JF; EURECA Investigator Study Group
Intensive Care Med 2023 May;49(5):517-529. Epub 2023 Apr 6 doi: 10.1007/s00134-023-07032-9. PMID: 37022378

Recent systematic reviews

Pichl T, Wedderburn CJ, Hoskote C, Turtle L, Bharucha T
Int J Infect Dis 2022 Jun;119:102-110. Epub 2022 Mar 10 doi: 10.1016/j.ijid.2022.03.010. PMID: 35283297
Knudtzen FC, Eikeland R, Bremell D, Quist-Paulsen E, Johansen IS, Solheim AM, Skarphédinsson S
Clin Microbiol Infect 2022 May;28(5):649-656. Epub 2021 Nov 10 doi: 10.1016/j.cmi.2021.11.001. PMID: 34768019
Goulenok T, Buzelé R, Duval X, Bruneel F, Stahl JP, Fantin B
Med Mal Infect 2017 May;47(3):206-220. Epub 2017 Mar 20 doi: 10.1016/j.medmal.2017.01.006. PMID: 28336304
Connolly MP, Goodwin E, Schey C, Zummo J
Pathog Glob Health 2017 Feb;111(1):31-44. Epub 2017 Jan 16 doi: 10.1080/20477724.2016.1273597. PMID: 28090819Free PMC Article
Khandaker G, Jung J, Britton PN, King C, Yin JK, Jones CA
Dev Med Child Neurol 2016 Nov;58(11):1108-1115. Epub 2016 Jul 16 doi: 10.1111/dmcn.13197. PMID: 27422743

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...