Rasmussen's encephalitis 2113730 219147583 2008-06-13T20:06:26Z 67.164.115.66 /* Associated conditions */ wikipedia {{Infobox_Disease | Name = {{PAGENAME}} | Image = | Caption = | DiseasesDB = 33757 | ICD10 = | ICD9 = {{ICD9|323.81}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = | }} '''Rasmussen's encephalitis''', also Chronic Focal Encephalitis (CFE), is a rare, progressive [[Neurology|neurological disorder]], characterized by frequent and severe [[seizure]]s, loss of [[motor skill]]s and speech, [[hemiparesis]] (paralysis on one side of the body), [[encephalitis]] (inflammation of the brain), [[dementia]], and mental deterioration. The disorder, which affects a single [[cerebral hemisphere]], generally occurs in children under the age of 10. ==Treatment== When seizures have not spontaneously remitted by the time [[hemiplegia]] and [[aphasia]] are complete, the standard treatment for Rasmussen's encephalitis is surgery to remove or disconnect the affected part of the brain ([[hemispherectomy]]). Although anti-epileptic [[psychoactive drug|drugs]] may be prescribed initially, they are usually not effective in controlling the seizures. Alternative treatments may include [[plasmapheresis]] (the removal and reinfusion of blood plasma), intravenous immunoglobulin,[[ketogenic diet]] (high fat, low carbohydrate), and [[steroids]]. ==Associated conditions== Rasmussen's encephalitis has been recorded with a [[neurovisceral]] [[porphyria]], [[acute intermittent porphyria]]. Drug resistant/drug refractory seizures, [[Lennox-Gastaut syndrome]] require deeper investigations for neurovisceral porphyrias including acute intermittent porphyria, [[hereditary coproporphyria]] and [[variegate porphyria]]. Multiple anti-seizure drugs are porphyrinogenic and should be avoided if these genetic disorders are present. Diagnosis may be difficult in children who require enzyme or DNA testing for these disorders. ==Prognosis== [[Prognosis]] for individuals with Rasmussen's encephalitis varies. Untreated, the disorder may lead to severe neurological deficits including [[mental retardation]] and paralysis. In some patients surgery decreases seizures. However, most patients are left with some paralysis and speech deficits. ==Eponym== It is named for Theodore Rasmussen.<ref>{{WhoNamedIt|synd|2828}}</ref><ref name="pmid13566382">{{cite journal |author=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=}}</ref> ==Genetics== In the 1990s, there was some evidence tying Rasmussen's encephalitis to [[GRIA3]] (GluR3)<ref name="pmid8036512">{{cite journal |author=Rogers SW, Andrews PI, Gahring LC, ''et al'' |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}}</ref>, but more recent studies have been inconclusive.<ref name="pmid15249609">{{cite journal |author=Watson R, Jiang Y, Bermudez I, ''et al'' |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 |doi=}}</ref> ==References== <references/> ==External links== * {{NINDS|rasmussen}} (Note: parts of this entry were copied from this [[Public Domain]] source.) [[Category:Encephalitis]] [[Category:Neurological disorders]] [[de:Rasmussen-Enzephalitis]] [[fr:Encéphalite focale de Rasmussen]] [[pl:Zapalenie mózgu Rasmussena]] [[fi:Rasmussenin aivokuume]]