Encephalitis lethargica 365012 220598154 2008-06-20T17:07:40Z DOI bot 6652755 Citation maintenance. You can [[WP:DOI|use this bot]] yourself! Please [[User:DOI_bot/bugs|report any bugs]]. {{DiseaseDisorder infobox | Name = Encephalitis lethargica | ICD10 = A85.8 | ICD9 = {{ICD9|049.8}} | }} '''Encephalitis lethargica''' (EL) or '''von Economo disease''' is an atypical form of [[encephalitis]]. Also known as "sleepy sickness" or as "sleeping sickness" (though different from the [[sleeping sickness]] transmitted by the [[tsetse fly]]), EL is a devastating illness that swept the world in the 1920s and then vanished as quickly as it had appeared. First described by the neurologist [[Constantin von Economo]] (1876-1931) in 1916, EL attacks the brain, leaving some victims in a statue-like condition, speechless and motionless.<ref name=Dale_2004>{{cite journal | author = Dale RC, Church AJ, Surtees RA, ''et al'' | title = Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity | journal = Brain | volume = 127 | issue = Pt 1 | pages = 21–33 | year = 2004 | url = http://brain.oxfordjournals.org/cgi/content/full/127/1/21 | pmid = 14570817 | doi = 10.1093/brain/awh008}}</ref> Between [[1917]] and [[1928]], an [[epidemic]] of encephalitis lethargica spread around the world; no recurrence of the epidemic has since been reported, though isolated cases continue to occur.<ref name=Stryker_1925>{{cite journal | author = Stryker Sue B. | title = Encephalitis lethargica: the behavior residuals | journal = Training School Bulletin | volume = 22 | issue = 1925 | pages = 152–7}}</ref><ref name=Reid_2001>{{cite journal | author = Reid AH, McCall S, Henry JM, Taubenberger JK | title = Experimenting on the past: the enigma of von Economo's encephalitis lethargica | journal = J. Neuropathol. Exp. Neurol. | volume = 60 | issue = 7 | pages = 663–70 | year = 2001 | pmid = 11444794 | doi = }}</ref> ==Symptoms== Encephalitis lethargica is characterized by high [[fever]], sore throat, [[headache]], [[Diplopia|double vision]], delayed physical and mental response, sleep inversion, [[catatonia]] and [[lethargy]].<ref name=Dale_2004/> In acute cases, patients may enter a [[coma]]-like state ([[akinetic mutism]]). Patients may also experience abnormal [[eye]] movements, [[parkinsonism]], upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including [[psychosis]]. [[Postencephalitic parkinsonism|Postencephalitic Parkinson's disease]] may develop after a bout of encephalitis, sometimes as long as a year after the start of the illness. ==Cause== The cause of encephalitis lethargica is not known for certain. However, research in 2004 suggested that the disease is due to an [[immune system|immune reaction]]. In this study, many of the people with encephalitis lethargica had experienced recent [[pharyngitis]] and the authors found some evidence linking the reaction to prior [[streptococcal pharyngitis|strep throat]]. They hypothesised that encephalitis lethargica, [[Sydenham's chorea]] and [[PANDAS]] (paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) are mediated by variations of the post-streptococcal immune response.<ref name=Dale_2004/> There is also some evidence of an [[autoimmune]] origin with antibodies (IgG) from patients with EL binding to neurons in the [[basal ganglia]] and [[mid-brain]]. ==Treatment== Treatment for encephalitis lethargica in the early stages is patient stabilization, which may be very difficult. There is little evidence so far of a consistent effective treatment for the initial stages, though some patients given [[steroids]] have seen improvement.<ref name=Blunt_1997>{{cite journal | author = Blunt SB, Lane RJ, Turjanski N, Perkin GD | title = Clinical features and management of two cases of encephalitis lethargica | journal = Mov. Disord. | volume = 12 | issue = 3 | pages = 354–9 | year = 1997 | pmid = 9159730 | doi = 10.1002/mds.870120314}}</ref> Other patients have been less fortunate, and the disease then becomes progressive, with evidence of brain damage similar to [[Parkinson's disease]]. Treatment is then symptomatic. [[Levodopa]] (<small>L</small>-DOPA) and other anti-parkinson drugs often produce dramatic responses. However in most of the patients who were given <small>L</small>-DOPA in the 1960s, the amelioration of the disease was short lived. The course of encephalitis lethargica varies depending upon complications or accompanying disorders. ==Popular culture== The discovery that <small>L</small>-DOPA could relieve some symptoms was described in the book ''[[Awakenings (book)|Awakenings]]'' by [[Oliver Sacks]] in [[1973]]. The book was used by [[Harold Pinter]] as the basis of his one-act play ''[[A Kind of Alaska]]'', performed in [[1982]] starring [[Judi Dench]]. ''[[Awakenings (film)|Awakenings]]'' is also the title of a 1990 movie starring [[Robin Williams]] based upon the book. The disease is also mentioned in [[Neil Gaiman]]'s [[The Sandman (Vertigo)|The Sandman]]. In this fictional comic book series, the disease is in some way caused by the capture and imprisonment of Morpheus, the King of Dreams. In his absence, people on Earth mysteriously fall prey to the sleeping sickness as he cannot be there to maintain and restore order. The disease is researched and mentioned in the Canadian television show [[ReGenesis]], in the last few episodes of the second series. It is also believed that in [[Salem witch trials]] the girls that were apparently [[bewitched]] could have possibly been affected by the disease. The visions of spirits could have been the double vision that is possible and the fits and tremors the girls experienced are all possible symptoms. The [[Big Black]] song, L Dopa, is based on Encephalitis Lethargica. ==References== <!-- --------------------------------------------------------------- See http://en.wikipedia.org/wiki/Wikipedia:Footnotes for a discussion of different citation methods and how to generate footnotes using the <ref> & </ref> tags and the {{Reflist}} template -------------------------------------------------------------------- --> {{Reflist}} ==External links== *[http://www.ninds.nih.gov/health_and_medical/disorders/encephalitis_lethargica.htm National Institute of Neurological Disorders and Stroke] *[http://news.bbc.co.uk/1/hi/health/3930727.stm Mystery of the Forgotten Plague]: BBC news item about the tracing of the infectious agent in encephalitis lethargica *[http://www.bbc.co.uk/sn/tvradio/programmes/horizon/broadband/tx/pandemic]: BBC Horizon programme suggesting linkage between encephalitis lethargica and Spanish flu pandemic *[http://www.bbc.co.uk/health/conditions/encephalitislethargica1.shtml BBC health article on encephalitis lethargica] {{Viral diseases}} [[Category:Encephalitis]] [[Category:Neurology]] [[Category:Unsolved problems in neuroscience]] [[bg:Летаргичен енцефалит]] [[de:Europäische Schlafkrankheit]] [[nl:Encephalitis lethargica]] [[pl:Zapalenie mózgu von Economo]] [[uk:Летаргічний енцефаліт]]