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| {{Infobox medical condition (new)
| | #REDIRECT [[Rasmussen syndrome]] |
| | name = Rasmussen's encephalitis
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| | synonyms = '''Chronic focal encephalitis'''
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| | image = CT scan Rasmussen's encephalitis.png
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| | caption = Brain CT scan of a girl with Rasmussen's encephalitis.
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| '''Rasmussen's encephalitis''', is a rare [[Inflammation|inflammatory]] [[Neurology|neurological]] disease, characterized by frequent and severe [[seizure]]s, loss of [[motor skill]]s and speech, [[hemiparesis]] (weakness on one side of the body), [[encephalitis]] (inflammation of the brain), and [[dementia]]. The illness affects a single [[cerebral hemisphere]] and generally occurs in children under the age of 15.
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| ==Signs and symptoms==
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| The condition mostly affects children, with an average age of 6 years. However, one in ten people with the condition develops it in adulthood.
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| There are two main stages, sometimes preceded by a 'prodromal stage' of a few months. In the ''acute stage'', lasting four to eight months, the inflammation is active and the symptoms become progressively worse. These include weakness of one side of the body ([[hemiparesis]]), loss of vision for one side of the [[visual field]] ([[hemianopia]]), and cognitive difficulties (affecting learning, memory or language, for example). Epileptic [[seizure]]s are also a major part of the illness, although these are often [[Seizure types#Partial seizures|partial]]. Focal motor seizures or [[epilepsia partialis continua]] are particularly common, and may be very difficult to control with drugs.
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| In the chronic or ''residual stage'', the inflammation is no longer active, but the sufferer is left with some or all of the symptoms because of the damage that the inflammation has caused. In the long term, most patients are left with some epilepsy, paralysis and cognitive problems, but the severity varies considerably.<ref name="pmid15689357"/>
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| == Pathophysiology ==
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| In Rasmussen’s encephalitis, there is [[chronic inflammation]] of the brain, with infiltration of [[T lymphocyte]]s into the brain tissue. In most cases, this affects only one [[cerebral hemisphere]], either the left or the right. This inflammation causes permanent damage to the cells of the brain, leading to [[atrophy]] of the hemisphere; the epilepsy that this causes may itself contribute to the brain damage. The epilepsy might derive from a disturbed GABA release,<ref>{{cite journal|last=Rassner|first=Michael P.|author2=van Velthoven-Wurster, Vera|author3=Ramantani, Georgia|author4=Feuerstein, Thomas J.|title=Altered transporter-mediated neocortical GABA release in Rasmussen encephalitis|journal=Epilepsia|date=March 2013|volume=54|issue=3|pages=e41–e44|doi=10.1111/epi.12093|pmid=23360283}}</ref> the main inhibitory neurotransmitter of the mammalian brain.
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| The cause of the inflammation is not known: infection by a [[virus]] has been suggested, but the evidence for this is inconclusive.<ref name="pmid15689357">{{cite journal |last = Bien |author2=Granata, Antozzi |title = Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement |journal = Brain |volume = 128 |pages = 454–471 |year = 2005 |doi = 10.1093/brain/awh415 |pmid = 15689357 |first1 = CG |issue = Pt 3 |display-authors = 1|doi-access = free }}</ref> In the 1990s it was suggested that [[auto-antibodies]] against the glutamate receptor [[GluR3]] were important in causing the disease,<ref name="pmid8036512">{{cite journal |author=Rogers SW |title=Autoantibodies to glutamate receptor GluR3 in Rasmussen's encephalitis |journal=Science |volume=265 |issue=5172 |pages=648–51 |year=1994 |pmid=8036512 |doi=10.1126/science.8036512 |name-list-format=vanc|author2=Andrews PI |author3=Gahring LC |display-authors=3 |last4=Whisenand |first4=T |last5=Cauley |first5=K |last6=Crain |first6=B |last7=Hughes |first7=T. |last8=Heinemann |first8=S. |last9=McNamara |first9=J.}}</ref> but this is no longer thought to be the case.<ref name="pmid15249609">{{cite journal |author=Watson R |title=Absence of antibodies to glutamate receptor type 3 (GluR3) in Rasmussen encephalitis |journal=Neurology |volume=63 |issue=1 |pages=43–50 |year=2004 |pmid=15249609 |name-list-format=vanc|author2=Jiang Y |author3=Bermudez I |display-authors=3 |last4=Houlihan |first4=L |last5=Clover |first5=L |last6=McKnight |first6=K |last7=Cross |first7=JH |last8=Hart |first8=IK |last9=Roubertie |first9=A |doi=10.1212/01.WNL.0000132651.66689.0F}}</ref> However, more recent studies report the presence of autoantibodies against the NMDA-type glutamate receptor subunit GluRepsilon2 (anti-NR2A antibodies) in a subset of patients with Rasmussen's encephalitis.<ref name="pmid15987271">{{cite journal |author=Takahashi Y |title=Autoantibodies and cell-mediated autoimmunity to NMDA-type GluRepsilon2 in patients with Rasmussen's encephalitis (RE) and chronic progressive epilepsia partialis continua |journal=Epilepsia |volume=46 |issue=Suppl 5 |pages=152–158 |year=2005 |pmid=15987271 |doi=10.1111/j.1528-1167.2005.01024.x |name-list-format=vanc|author2=Mori H |author3=Mishina M |display-authors=3 |last4=Watanabe |first4=Masahiko |last5=Kondo |first5=Naomi |last6=Shimomura |first6=Jiro |last7=Kubota |first7=Yuko |last8=Matsuda |first8=Kazumi |last9=Fukushima |first9=Katsuyuki}}</ref> There has also been some evidence that patients suffering from RE express auto-antibodies against alpha 7 subunit of the [[CHRNA7|nicotinic acetylcholine receptor]].<ref>{{cite journal|last=Watson|first=R |author2=Jepson, JE |author3=Bermudez, I |author4=Alexander, S |author5=Hart, Y |author6=McKnight, K |author7=Roubertie, A |author8=Fecto, F |author9=Valmier, J |author10=Sattelle, DB |author11=Beeson, D |author12=Vincent, A |author13=Lang, B|title=Alpha7-acetylcholine receptor antibodies in two patients with Rasmussen encephalitis.|journal=Neurology|date=Dec 13, 2005|volume=65|issue=11|pages=1802–4|pmid=16344526|doi=10.1212/01.wnl.0000191566.86977.04}}</ref> By sequencing T cell receptors from various compartments it could be shown that RE patients present with peripheral CD8+ T-cell expansion which in some cases have been proven for years after disease onset.<ref>{{cite journal |vauthors=Schneider-Hohendorf T, Mohan H, Bien CG, Breuer J, Becker A, Görlich D, Kuhlmann T, Widman G, Herich S, Elpers C, Melzer N, Dornmair K, Kurlemann G, Wiendl H, Schwab N |title=CD8(+) T-cell pathogenicity in Rasmussen encephalitis elucidated by large-scale T-cell receptor sequencing |journal=Nat Commun |volume=7 |issue= |pages=11153 |year=2016 |pmid=27040081 |pmc=4822013 |doi=10.1038/ncomms11153 }}</ref>
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| Rasmussen's encephalitis has been recorded with a [[neurovisceral]] [[porphyria]], and [[acute intermittent porphyria]].<ref>{{cite journal |vauthors=Tziperman B, Garty BZ, Schoenfeld N, Hoffer V, Watemberg N, Lev D, Ganor Y, Levite M, Lerman-Sagie T |title=Acute intermittent porphyria, Rasmussen encephalitis, or both? |journal=J. Child Neurol. |volume=22 |issue=1 |pages=99–105 |year=2007 |pmid=17608316 |doi=10.1177/0883073807299962 }}</ref>
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| <!-- None of the "causes" proposed explains why the disease only affects one hemisphere of the brain. -->
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| ==Diagnosis==
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| [[File:Rasmussen Brainbiopsy CD8.jpg|thumb|[[Brain biopsy]] in Rasmussen's encephalitis showing lymphocytic infiltrates staining for [[CD8]] on [[immunohistochemistry]]]]
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| [[File:MRI Rasmussen's encephalitis.png|thumb|Brain MRI of an 8-year-old female with Rasmussen's encephalitis.<br />'''Left''': December 2008, the patient was presented with headache and ''epilepsia partialis continua''. There are lesions with local brain swelling in the right parietal and occipital lobes and right cerebellar hemisphere.<br />'''Right''': April 2009, the same patient, now she is comatose with ''epilepsia partialis continua''. There is progression of the encephalitis - the left cerebral hemisphere has been involved with severe brain swelling and shift of the midline structures.]]
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| The diagnosis may be made on the clinical features alone, along with tests to rule out other possible causes. An [[EEG]] will usually show the electrical features of epilepsy and slowing of brain activity in the affected hemisphere, and [[MRI]] brain scans will show gradual shrinkage of the affected hemisphere with signs of inflammation or scarring.<ref name="pmid24457189"/>
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| [[Brain biopsy]] can provide very strong confirmation of the diagnosis, but this is not always necessary.<ref name="pmid24457189"/><ref name="pmid26941743">{{cite journal |vauthors=Owens GC, Chang JW, Huynh MN, Chirwa T, Vinters HV, Mathern GW |title=Evidence for Resident Memory T Cells in Rasmussen Encephalitis |journal=Front Immunol |volume=7 |issue= |pages=64 |year=2016 |pmid=26941743 |pmc=4763066 |doi=10.3389/fimmu.2016.00064 }}</ref>
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| ==Treatment==
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| During the acute stage, treatment is aimed at reducing the inflammation. As in other inflammatory diseases, [[steroids]] may be used first of all, either as a short course of high-dose treatment, or in a lower dose for long-term treatment. [[Intravenous immunoglobulin]] is also effective both in the short term and in the long term, particularly in adults where it has been proposed as first-line treatment.<ref>{{Cite journal |last = Hart |author2=Cortez, Andermann |author3=Hwang, Fish |title = Medical treatment of Rasmussen syndrome (chronic encephalitis and epilepsy): effect of high-dose steroids or immunoglobulins in 19 patients |journal = Neurology |volume = 44 |pages = 1030–1036 |year = 1994 |id = 8208394 |pmid = 8208394 |first1 = YM |issue = 6 |doi = 10.1212/WNL.44.6.1030}}</ref> Other similar treatments include [[plasmapheresis]] and [[tacrolimus]], though there is less evidence for these. None of these treatments can prevent permanent disability from developing.<ref name="pmid24457189">{{cite journal |vauthors=Varadkar S, Bien CG, Kruse CA, Jensen FE, Bauer J, Pardo CA, Vincent A, Mathern GW, Cross JH |title=Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances |journal=Lancet Neurol |volume=13 |issue=2 |pages=195–205 |year=2014 |pmid=24457189 |pmc=4005780 |doi=10.1016/S1474-4422(13)70260-6 }}</ref><ref name="pmid23433490">{{cite journal |vauthors=Takahashi Y, Yamazaki E, Mine J, Kubota Y, Imai K, Mogami Y, Baba K, Matsuda K, Oguni H, Sugai K, Ohtsuka Y, Fujiwara T, Inoue Y |title=Immunomodulatory therapy versus surgery for Rasmussen syndrome in early childhood |journal=Brain Dev. |volume=35 |issue=8 |pages=778–85 |year=2013 |pmid=23433490 |doi=10.1016/j.braindev.2013.01.010 }}</ref>
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| During the residual stage of the illness when there is no longer active inflammation, treatment is aimed at improving the remaining symptoms. Standard anti-epileptic drugs are usually ineffective in controlling seizures, and it may be necessary to surgically remove or disconnect the affected cerebral hemisphere, in an operation called [[hemispherectomy]] or via a corpus callosotomy. This usually results in further weakness, [[hemianopsia]] and cognitive problems, but the other side of the brain may be able to take over some of the function, particularly in young children. The operation may not be advisable if the left hemisphere is affected, since this hemisphere contains most of the parts of the brain that control language. However, hemispherectomy is often very effective in reducing seizures.<ref name="pmid15689357"/><ref name="pmid24457189"/>
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| ==History==
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| It is named for the [[neurosurgeon]] {{ill|Theodore Rasmussen|de}} (1910–2002), who succeeded [[Wilder Penfield]] as head of the [[Montreal Neurological Institute and Hospital|Montreal Neurological Institute]], and served as Neurosurgeon-in-Chief at the Royal Victoria Hospital.<ref>{{WhoNamedIt|synd|2828|Rasmussen's encephalitis}}</ref><ref name="pmid13566382">{{cite journal |vauthors=Rasmussen T, Olszewski J, Lloyd-Smith D |title=Focal seizures due to chronic localized encephalitis |journal=Neurology |volume=8 |issue=6 |pages=435–45 |year=1958 |pmid=13566382 |doi=10.1212/WNL.8.6.435|doi-access=free }}</ref>
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| ==Society==
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| The [[Hemispherectomy Foundation]] was formed in 2008 to assist families with children who have Rasmussen's Encephalitis and other conditions that require hemispherectomy.<ref>{{cite web|title=The Community News |url=http://www.community-news.com/node/3302 |accessdate=2009-02-25 |url-status=dead |archiveurl=https://web.archive.org/web/20090329081810/http://www.community-news.com/node/3302 |archivedate=March 29, 2009 }}</ref>
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| [http://rechildrens.com/ The RE Children's Project] was founded in 2010 to increase awareness of Rasmussen's Encephalitis. Its primary purpose is to support scientific research directed toward finding a cure for this disease.
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| ==References==
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| {{Reflist}}
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| == External links ==
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| {{Medical resources
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| | DiseasesDB = 33757
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| | ICD10 = {{ICD10|G|04|8|g|04}}
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| | ICD9 = {{ICD9|323.81}}
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| }} | | }} |
| * {{NINDS|Rasmussen}} (Note: parts of this entry were copied from this [[Public Domain]] source.)
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| [[Category:Encephalitis]]
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| [[Category:Disorders causing seizures]]
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| [[Category:Epilepsy types]]
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