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{{See also|Acute disseminated encephalomyelitis}}{{Infobox medical condition |
{{See also|Acute disseminated encephalomyelitis}}{{Infobox medical condition |
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| name = Necrotizing encephalopathy |
| name = Necrotizing encephalopathy |
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| synonyms = Acute necrotizing encephalitis, Acute necrotizing encephalopathy of childhood (ANEC), ANE, infection-induced acute encephalopathy |
| synonyms = Acute necrotizing encephalitis, Acute necrotizing encephalopathy of childhood (ANEC), ANE, infection-induced acute encephalopathy (IIAE), ADANE, ANE1 |
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'''Acute necrotizing encephalopathy''' |
'''Acute necrotizing encephalopathy''' ('''ANE''' or '''ANE''') or sometimes '''necrotizing encephalitis''', is a rare type of brain disease ([[encephalopathy]]) that occurs following a viral infection.<ref name=":0">{{Cite web |date=2022-06-16 |title=Acute necrotizing encephalopathy - National Organization for Rare Disorders |url=https://rarediseases.org/gard-rare-disease/acute-necrotizing-encephalopathy/ |access-date=2023-03-05 |website=rarediseases.org |language=en-US}}</ref> Most commonly, it develops secondary to infection with [[Influenza A virus|influenza A]], [[Influenza B virus|influenza B]], and the [[Human herpesvirus 6|human herpes virus 6]].<ref name=":1">{{Cite web |title=Acute necrotizing encephalopathy - About the Disease - Genetic and Rare Diseases Information Center |url=https://rarediseases.info.nih.gov/diseases/13233/acute-necrotizing-encephalopathy |access-date=2023-03-05 |website=rarediseases.info.nih.gov |language=en}}</ref> Multiple subtypes, associated with specific genes have been found and are termed '''infection-induced acute encephalopathies''' ('''IIAE'''). |
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== Symptoms == |
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Acute necrotizing encephalopathy type 1 typically appears in infancy or early childhood, although some people do not develop the condition until adolescence or adulthood. People with this condition usually show typical symptoms of an infection, such as fever, cough, congestion, vomiting, and diarrhea, for a few days. Following these flu-like symptoms, affected individuals develop neurological problems, such as seizures, hallucinations, difficulty coordinating movements ([[ataxia]]), or abnormal muscle tone. Eventually, most affected individuals go into a coma, which usually lasts for a number of weeks. The condition is described as "acute" because the episodes of illness are time-limited.<ref name="MedLine" /> |
Acute necrotizing encephalopathy type 1 typically appears in infancy or early childhood, although some people do not develop the condition until adolescence or adulthood. People with this condition usually show typical symptoms of an infection, such as fever, cough, congestion, vomiting, and diarrhea, for a few days. Following these flu-like symptoms, affected individuals develop neurological problems, such as seizures, hallucinations, difficulty coordinating movements ([[ataxia]]), or abnormal muscle tone. Eventually, most affected individuals go into a coma, which usually lasts for a number of weeks. The condition is described as "acute" because the episodes of illness are time-limited.<ref name="MedLine" /> |
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People with acute necrotizing encephalopathy type 1 develop areas of damages ([[Lesion|lesions]]) in certain regions of the brain. As the condition progresses, these brain regions develop swelling ([[edema]]), bleeding ([[hemorrhage]]), and then tissue death ([[necrosis]]). The progressive brain damage and tissue loss results in encephalopathy.<ref name="MedLine" /> |
People with acute necrotizing encephalopathy type 1 develop areas of damages ([[Lesion|lesions]]) in certain regions of the brain. As the condition progresses, these brain regions develop swelling ([[edema]]), bleeding ([[hemorrhage]]), and then tissue death ([[necrosis]]). The progressive brain damage and tissue loss results in encephalopathy.<ref name="MedLine" /> |
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== Prognosis == |
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Approximately one-third of individuals with acute necrotizing encephalopathy type 1 do not survive their illness and subsequent neurological decline. Of those who do survive, about half have permanent brain damage due to tissue necrosis, resulting in impairments in walking, speech, and other basic functions. Over time, many of these skills may be regained, but the loss of brain tissue is permanent. Other individuals who survive their illness appear to recover completely.<ref name="MedLine" /> |
Approximately one-third of individuals with acute necrotizing encephalopathy type 1 do not survive their illness and subsequent neurological decline. Of those who do survive, about half have permanent brain damage due to tissue necrosis, resulting in impairments in walking, speech, and other basic functions. Over time, many of these skills may be regained, but the loss of brain tissue is permanent. Other individuals who survive their illness appear to recover completely.<ref name="MedLine" /> |
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|website=[[MedlinePlus]] |publisher=[[United States National Library of Medicine]] |access-date=June 27, 2022 |url=https://medlineplus.gov/genetics/condition/acute-necrotizing-encephalopathy-type-1/}} {{PD-notice}}</ref> |
|website=[[MedlinePlus]] |publisher=[[United States National Library of Medicine]] |access-date=June 27, 2022 |url=https://medlineplus.gov/genetics/condition/acute-necrotizing-encephalopathy-type-1/}} {{PD-notice}}</ref> |
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== History == |
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The first case was described in 1955 by Belgian neurologist Ludo van Bogaert.<ref>{{Cite journal |last=Van Bogaert |first=L. |last2=Radermecker |first2=J. |last3=Devos |first3=J. |date=1955 |title=[A fatal case of acute necrosing encephalitis; its position with reference to the group of encephalites transmitted by arthropods and to herpetic encephalitis] |url=https://pubmed.ncbi.nlm.nih.gov/13281224 |journal=Revue Neurologique |volume=92 |issue=5 |pages=329–356 |issn=0035-3787 |pmid=13281224}}</ref> |
The first case in the medical litterature was described in 1955 by Belgian neurologist Ludo van Bogaert.<ref>{{Cite journal |last=Van Bogaert |first=L. |last2=Radermecker |first2=J. |last3=Devos |first3=J. |date=1955 |title=[A fatal case of acute necrosing encephalitis; its position with reference to the group of encephalites transmitted by arthropods and to herpetic encephalitis] |url=https://pubmed.ncbi.nlm.nih.gov/13281224 |journal=Revue Neurologique |volume=92 |issue=5 |pages=329–356 |issn=0035-3787 |pmid=13281224}}</ref> |
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== Genetics == |
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ANE can be familial or sporadic, but both forms are very similar to each other. Most familial cases are caused by genetic changes in the [[RANBP2]] gene, and are known as infection-induced acute encephalopathy 3 (IIAE3).<ref name=":1" /> |
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In addition there is acute infection-induced (herpes-specific) encephalopathy-1 (IIAE1) associated with a homozygous [[UNC93B1]] gene; and herpes-specific IIAE2 associated with the TLR3 gene;<ref>{{Cite web |title=Entry |
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- *603029 - TOLL-LIKE RECEPTOR 3; TLR3 |
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- OMIM |url=https://omim.org/entry/603029 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> as well as IIAE4, associated with the [[CPT2 (gene)|CPT2]] gene;<ref>{{Cite web |title=Entry |
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- OMIM |url=https://omim.org/entry/614212 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> herpes-specific IIAE5 associated with the TRAF3 gene;<ref>{{Cite web |title=Entry |
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- #614849 - ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED (HERPES-SPECIFIC), SUSCEPTIBILITY TO, 5; IIAE5 |
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- OMIM |url=https://omim.org/entry/614849 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> herpes-specific IIAE6 associated with the TICAM1 gene;<ref>{{Cite web |title=Entry |
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- #614850 - ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED (HERPES-SPECIFIC), SUSCEPTIBILITY TO, 6; IIAE6 |
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- OMIM |url=https://omim.org/entry/614850 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> herpes-specific IIAE7 associated with the IRF3 gene;<ref>{{Cite web |title=Entry |
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- #616532 - ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED (HERPES-SPECIFIC), SUSCEPTIBILITY TO, 7; IIAE7 |
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- OMIM |url=https://omim.org/entry/616532 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> herpes-specific IIAE8 associated with the TBK1 gene;<ref>{{Cite web |title=Entry |
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- #617900 - ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED (HERPES-SPECIFIC), SUSCEPTIBILITY TO, 8; IIAE8 |
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- OMIM |url=https://omim.org/entry/617900 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> IIAE9 associated with the NUP214 gene;<ref>{{Cite web |title=Entry |
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- #618426 - ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED, SUSCEPTIBILITY TO, 9; IIAE9 |
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- OMIM |url=https://omim.org/entry/618426 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> herpes-specific IIAE10 associated with the SNORA31 gene;<ref>{{Cite web |title=Entry |
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- #619396 - ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED (HERPES-SPECIFIC), SUSCEPTIBILITY TO, 10; IIAE10 |
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- OMIM |url=https://omim.org/entry/619396 |access-date=2023-03-05 |website=omim.org |language=en-us}}</ref> and herpes-specific IIAE11 associated with the DBR1 gene.<ref>{{Cite web |title=Entry |
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- #619441 - ENCEPHALITIS, ACUTE, INFECTION (VIRAL)-INDUCED, SUSCEPTIBILITY TO, 11; IIAE11 |
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== Treatment == |
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Optimal treatment regimes for the condition are not know. Suggested treatments may be [[Corticosteroid|corticosteroids]] and [[cytokine]] regulators such as [[TNF inhibitor]]<nowiki/>s.<ref name=":0" /> |
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==References== |
==References== |
Revision as of 09:25, 5 March 2023
Necrotizing encephalopathy | |
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Other names | Acute necrotizing encephalitis, Acute necrotizing encephalopathy of childhood (ANEC), ANE, infection-induced acute encephalopathy (IIAE), ADANE, ANE1 |
Specialty | Infectious disease Neurology. |
Symptoms | fever, cough, congestion, vomiting and diarrhea, for several days. After these flu-like symptoms, the affected individuals develop neurological problems, such as seizures, hallucinations, difficulty coordinating movements (ataxia) or abnormal muscle tone.[1] |
Causes | Influenza is the most common virus found in people with acute necrotizing encephalopathy type 1; other viruses that are known to trigger this condition include human herpesvirus 6, coxsackie virus, and enteroviruses. In rare cases, the bacterium Mycoplasma pneumoniae is involved.[1] |
Prevention | Vaccination,[2] face coverings, quarantine, physical/social distancing, ventilation, hand washing[3] |
Medication | Steroid and intravenous immunoglobulin (IVIG) |
Prognosis | Approximately one-third of individuals with acute necrotizing encephalopathy type 1 do not survive their illness and subsequent neurological decline. Of those who do survive, about half have permanent brain damage due to tissue necrosis, resulting in impairments in walking, speech, and other basic functions, there may also be permanent brain damage.[1] |
Frequency | At least 59 cases of this condition have been reported in the scientific literature[1] |
Acute necrotizing encephalopathy (ANE or ANE) or sometimes necrotizing encephalitis, is a rare type of brain disease (encephalopathy) that occurs following a viral infection.[4] Most commonly, it develops secondary to infection with influenza A, influenza B, and the human herpes virus 6.[5] Multiple subtypes, associated with specific genes have been found and are termed infection-induced acute encephalopathies (IIAE).
Symptoms
Acute necrotizing encephalopathy type 1 typically appears in infancy or early childhood, although some people do not develop the condition until adolescence or adulthood. People with this condition usually show typical symptoms of an infection, such as fever, cough, congestion, vomiting, and diarrhea, for a few days. Following these flu-like symptoms, affected individuals develop neurological problems, such as seizures, hallucinations, difficulty coordinating movements (ataxia), or abnormal muscle tone. Eventually, most affected individuals go into a coma, which usually lasts for a number of weeks. The condition is described as "acute" because the episodes of illness are time-limited.[1]
People with acute necrotizing encephalopathy type 1 develop areas of damages (lesions) in certain regions of the brain. As the condition progresses, these brain regions develop swelling (edema), bleeding (hemorrhage), and then tissue death (necrosis). The progressive brain damage and tissue loss results in encephalopathy.[1]
Prognosis
Approximately one-third of individuals with acute necrotizing encephalopathy type 1 do not survive their illness and subsequent neurological decline. Of those who do survive, about half have permanent brain damage due to tissue necrosis, resulting in impairments in walking, speech, and other basic functions. Over time, many of these skills may be regained, but the loss of brain tissue is permanent. Other individuals who survive their illness appear to recover completely.[1]
It is estimated that half of individuals with acute necrotizing encephalopathy type 1 are susceptible to recurrent episodes and will have another infection that results in neurological decline; some people may have numerous episodes throughout their lives. Neurological function worsens following each episode as more brain tissue is damaged.[1]
History
The first case in the medical litterature was described in 1955 by Belgian neurologist Ludo van Bogaert.[6]
Genetics
ANE can be familial or sporadic, but both forms are very similar to each other. Most familial cases are caused by genetic changes in the RANBP2 gene, and are known as infection-induced acute encephalopathy 3 (IIAE3).[5]
In addition there is acute infection-induced (herpes-specific) encephalopathy-1 (IIAE1) associated with a homozygous UNC93B1 gene; and herpes-specific IIAE2 associated with the TLR3 gene;[7] as well as IIAE4, associated with the CPT2 gene;[8] herpes-specific IIAE5 associated with the TRAF3 gene;[9] herpes-specific IIAE6 associated with the TICAM1 gene;[10] herpes-specific IIAE7 associated with the IRF3 gene;[11] herpes-specific IIAE8 associated with the TBK1 gene;[12] IIAE9 associated with the NUP214 gene;[13] herpes-specific IIAE10 associated with the SNORA31 gene;[14] and herpes-specific IIAE11 associated with the DBR1 gene.[15]
Treatment
Optimal treatment regimes for the condition are not know. Suggested treatments may be corticosteroids and cytokine regulators such as TNF inhibitors.[4]
References
- ^ a b c d e f g h "Acute necrotizing encephalopathy type 1". MedlinePlus. United States National Library of Medicine. Retrieved June 27, 2022. This article incorporates text from this source, which is in the public domain.
- ^ "COVID-19 vaccines". World Health Organization (WHO). Retrieved 3 March 2021.
- ^ Talic, S; Shah, S; Wild, H; Gasevic, D; Maharaj, A; Ademi, Z; Li, X; Xu, W; Mesa-Eguiagaray, I; Rostron, J; Theodoratou, E (17 November 2021). "Effectiveness of public health measures in reducing the incidence of covid-19, SARS-CoV-2 transmission, and covid-19 mortality: systematic review and meta-analysis". BMJ (Clinical Research Ed.). 375: e068302. doi:10.1136/bmj-2021-068302. ISSN 1756-1833. PMC 9423125. PMID 34789505. S2CID 244271780.
- ^ a b "Acute necrotizing encephalopathy - National Organization for Rare Disorders". rarediseases.org. 2022-06-16. Retrieved 2023-03-05.
- ^ a b "Acute necrotizing encephalopathy - About the Disease - Genetic and Rare Diseases Information Center". rarediseases.info.nih.gov. Retrieved 2023-03-05.
- ^ Van Bogaert, L.; Radermecker, J.; Devos, J. (1955). "[A fatal case of acute necrosing encephalitis; its position with reference to the group of encephalites transmitted by arthropods and to herpetic encephalitis]". Revue Neurologique. 92 (5): 329–356. ISSN 0035-3787. PMID 13281224.
- ^ "Entry - *603029 - TOLL-LIKE RECEPTOR 3; TLR3 - OMIM". omim.org. Retrieved 2023-03-05.
{{cite web}}
: line feed character in|title=
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{{cite web}}
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{{cite web}}
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{{cite web}}
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{{cite web}}
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{{cite web}}
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{{cite web}}
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{{cite web}}
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{{cite web}}
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