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07-SEPTEMBER-2008 03:17:44 - Encephalitis lethargica Encephalitis lethargica Classification and external resources Constantin von Economo ICD-10 A85.8 ICD-9 049.8 DiseasesDB 32498 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 1917,12 EL attacks the brain, leaving some victims in a statue-like condition, speechless and motionless.3 Between 1915 and 1926,4 an epidemic of encephalitis lethargica spread around the world; no recurrence of the epidemic has since been reported, though isolated cases continue to occur.56 Contents 1 Symptoms 2 Cause 3 Treatment 4 Popular culture 5 References 6 External links Symptoms Encephalitis lethargica. Its sequelae and treatment - Constantin Von Economo, 1931: front page Encephalitis lethargica. Its sequelae and treatment - Constantin Von Economo, 1931: front page L-DOPA L-DOPA Awakenings Awakenings Encephalitis lethargica is characterized by high fever, sore throat, headache, double vision, delayed physical and mental response, sleep inversion, catatonia and lethargy.3 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 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 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 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.3 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.7 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. Popular culture The discovery that L-DOPA could relieve some symptoms was described in the 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 is also the title of a 1990 movie starring Robin Williams based 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 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.8 References ^ synd/3356 at Who Named It ^ K. von Economo. Encepahlitis lethargica. Wiener klinische Wochenschrift, May 10, 1917, 30: 581-585. Die Encephalitis lethargica. Leipzig and Vienna, Franz Deuticke, 1918. ^ a b c Dale RC, Church AJ, Surtees RA, et al 2004. Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity. Brain 127 Pt 1: 21-33. doi:10.1093/brain/awh008. PMID 14570817. ^ Encephalitis lethargica at Dorland's Medical Dictionary ^ Stryker Sue B.. Encephalitis lethargica: the behavior residuals. Training School Bulletin 22 1925: 152-7. ^ Reid AH, McCall S, Henry JM, Taubenberger JK 2001. Experimenting on the past: the enigma of von Economo's encephalitis lethargica. J. Neuropathol. Exp. Neurol. 60 7: 663-70. PMID 11444794. ^ Blunt SB, Lane RJ, Turjanski N, Perkin GD 1997. Clinical features and management of two cases of encephalitis lethargica. Mov. Disord. 12 3: 354-9. doi:10.1002/mds.870120314. PMID 9159730. ^ Carlson, Laurie Winn. A Fever in Salem: A New Interpretation of the New England Witch Trials, Chicago, Ivan R. Dee, 1999. ISBN 978-1566633093 External links National Institute of Neurological Disorders and Stroke Mystery of the Forgotten Plague: BBC news item about the tracing of the infectious agent in encephalitis lethargica 1: BBC Horizon programme suggesting linkage between encephalitis lethargica and Spanish flu pandemic BBC health article on encephalitis lethargica v d e Infectious diseases - Virus diseases A80-B34, 042-079 CNS Encephalitis/ meningitis DNA virus: Progressive multifocal leukoencephalopathy RNA virus: Subacute sclerosing panencephalitis - Lymphocytic choriomeningitis - Tick-borne meningoencephalitis unknown: Encephalitis lethargica Eye DNA virus: Cytomegalovirus retinitis Other RNA virus: Rabies - Myelitis: Poliomyelitis Post-polio syndrome - Tropical spastic paraparesis Skin and mucous membrane lesions DNA virus, Herpesviridae: Herpes simplex - Chickenpox - Herpes zoster - KSHV DNA virus, other: Poxviridae Smallpox, Monkeypox, Cowpox, Vaccinia, Molluscum contagiosum - exanthem Roseola, Fifth disease - HPV Wart RNA virus: exanthem Measles, Rubella - picornavirus Hand, foot and mouth disease, Foot-and-mouth disease Digestive system Hepatitis DNA virus: B RNA virus: A - D - C - E - G Gastroenteritis DNA virus: Adenovirus RNA virus: Rotavirus - Norovirus - Astrovirus - Coronavirus Respiratory system RNA virus, IV: Acute viral nasopharyngitis - Severe acute respiratory syndrome RNA virus, V: Influenza/Avian influenza - Human parainfluenza viruses - RSV - hMPV Other/varied: Infectious mononucleosis - Viral pneumonia Sexually transmitted DNA virus: HPV Genital warts, Cervical cancer RNA virus, retrovirus: HIV AIDS, AIDS dementia complex - Adult T-cell leukemia Oncovirus DNA virus: Hepatitis B - HPV - Kaposi's sarcoma-associated herpesvirus RNA virus: Hepatitis C - HTLV Systemic DNA virus: Cytomegalovirus RNA virus: Mumps - Bornholm disease - Coxsackie B Retrieved from http://en..org/wiki/Encephalitis_lethargica Categories: Encephalitis | Neurology | Unsolved problems in neuroscience Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages БългарÑ?ки Deutsch Nederlands Polski УкраїнÑ?ька This page was last modified on 28 August 2008, at 02:02
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