Name = PAGENAME
Constantin von Economo
DiseasesDB = 32498
ICD10 = ICD10|A|85|8|a|80
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 sicknesstransmitted 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 1917, [WhoNamedIt|synd|3356] [K. von Economo. Encepahlitis lethargica. Wiener klinische Wochenschrift, May 10, 1917, 30: 581-585. Die Encephalitis lethargica. Leipzig and Vienna, Franz Deuticke, 1918.] EL attacks the brain, leaving some victims in a statue-like condition, speechless and motionless.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] Between 1915 and 1926, [DorlandsDict|nine/000952335|Encephalitis lethargica] an epidemicof encephalitis lethargica spread around the world; no recurrence of the epidemic has since been reported, though isolated cases continue to occur.cite journal | author = Stryker Sue B. | title = Encephalitis lethargica: the behavior residuals | journal = Training School Bulletin | volume = 22 | issue = 1925 | pages = 152–7] 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 = ]
Encephalitis lethargica is characterized by high
fever, sore throat, headache, double vision, delayed physical and mental response, sleep inversion, catatoniaand lethargy. In acute cases, patients may enter a coma-like state ( akinetic mutism). Patients may also experience abnormal eyemovements, parkinsonism, upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including psychosis.
Postencephalitic Parkinson's disease may develop after a bout of encephalitis, sometimes as long as a year after the start of the illness.
The cause of encephalitis lethargica is not known for certain. However, research in 2004 suggested that the disease is due to an immune reaction. In this study, many of the people with encephalitis lethargica had experienced recent
pharyngitisand the authors found some evidence linking the reaction to prior strep throat. They hypothesised that encephalitis lethargica, Sydenham's choreaand PANDAS(paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) are mediated by variations of the post-streptococcal immune response. There is also some evidence of an autoimmuneorigin with antibodies (IgG) from patients with EL binding to neurons in the basal gangliaand mid-brain.
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
steroidshave seen improvement.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] 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(L-DOPA) and other anti-parkinson drugs often produce dramatic responses. However in most of the patients who were given L-DOPA in the 1960s, the amelioration of the disease was short lived.
The course of encephalitis lethargica varies depending upon complications or accompanying disorders.
The discovery that L-DOPA could relieve some symptoms was described in the book "Awakenings" by
Oliver Sacksin 1973. The book was used by Harold Pinteras the basis of his one-act play " A Kind of Alaska", performed in 1982 starring Judi Dench. "Awakenings" is also the title of a 1990 movie starring Robin Williamsbased on the book.
The disease is also mentioned in
Neil Gaiman's 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 during the
Salem witch trialsthe girls that were apparently bewitchedcould 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. [Carlson, Laurie Winn. "A Fever in Salem: A New Interpretation of the New England Witch Trials", Chicago, Ivan R. Dee, 1999. ISBN 978-1566633093]
* [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]
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Look at other dictionaries:
encephalitis lethargica — [li thär′ji kə] n. a form of encephalitis, or sleeping sickness, epidemic in the period from 1915 to 1926 * * * … Universalium
encephalitis lethargica — [li thär′ji kə] n. a form of encephalitis, or sleeping sickness, epidemic in the period from 1915 to 1926 … English World dictionary
Encephalitis lethargica — Die Europäische Schlafkrankheit (Encephalitis lethargica) ist eine bereits von Hippokrates beschriebene Form der Gehirnentzündung, welche Lethargie, unkontrollierte Schlafanfälle und eine temporäre, der Parkinson Krankheit ähnliche Störung… … Deutsch Wikipedia
encephalitis lethargica — encephalitis le·thar·gi·ca li thär ji kə, le n epidemic virus encephalitis in which somnolence is marked called also lethargic encephalitis … Medical dictionary
encephalitis lethargica — noun an encephalitis that was epidemic between 1915 and 1926; symptoms include paralysis of the extrinsic eye muscle and extreme muscular weakness • Syn: ↑sleeping sickness, ↑sleepy sickness, ↑epidemic encephalitis, ↑lethargic encephalitis •… … Useful english dictionary
encephalitis lethargica — [lɪ θα:dʒɪkə] noun a form of encephalitis caused by a virus and characterized by headache and drowsiness leading to coma … English new terms dictionary
encephalitis lethargica — /ɛnˌsɛfəlaɪtəs ləˈθadʒɪkə/ (say en.sefuhluytuhs luh thahjikuh) noun → sleeping sickness (def. 2) … Australian English dictionary
Encephalitis — is an acute inflammation of the brain, commonly caused by a viral infection. It can be caused by a bacterial infection such as bacterial meningitis spreading directly to the brain (primary encephalitis), or may be a complication of a current… … Wikipedia
encephalitis le|thar|gi|ca — «luh THAHR juh kuh», = lethargic encephalitis. (Cf. ↑lethargic encephalitis) ╂[< New Latin encephalitis lethargica] … Useful english dictionary
lethargica — see ENCEPHALITIS LETHARGICA … Medical dictionary