Encephalitis lethargica and influenza. 1918 influenza, encephalitis lethargica, parkinsonism. [24], Many surviving patients of the 1915–1926 pandemic seemed to make a complete recovery and return to their normal lives. Encephalitis Lethargica was a devastating illness that swept the world in the 1920's. Would you like email updates of new search results? 1 The number affected during the epidemic is unknown, although estimated at more than one million cases. In particular, many of the patients presented with marked lethargy. 1997 Dec;90(12):709-10. 1982 Oct 16;2(8303):860-4 It left millions orphaned and medical science desperate to find its cause. The exact number of people infected is unknown, but it is estimated that more than one million people contracted the disease during the epidemic, which directly caused more than 500,000 deaths. The pandemic disappeared in 1927, as abruptly and mysteriously as it first appeared. Trans Stud Coll Physicians Phila. Proc R Soc Med. While many scientists were convinced that encephalitis lethargica was a disease of the past, trapped somewhere between the pages of history, virologist Professor John Oxford thought that the game was not yet over. [16] Postencephalitic parkinsonism was clearly documented to have followed an outbreak of encephalitis lethargica following the 1918 influenza pandemic; evidence for viral causation of the Parkinson's symptoms is circumstantial (epidemiologic, and finding influenza antigens in encephalitis lethargica patients), while evidence arguing against this cause is of the negative sort (e.g., lack of viral RNA in postencephalitic parkinsonian brain material). The third symptom would be recovery, followed by a Parkinson-like syndrome. In the hardest hit societies, everything else was put aside in a bid to cope with its ravages. The contemporaneous relationship of the 2 diseases led to speculation that they were causally related. Encephalitis lethargica emerged at around the same time and affected more than one million individuals. Although clinicopathologically unrelated, one such devastating epidemic was encephalitis lethargica (EL), known as “sleeping sickness” that coincided with the influenza epidemic of 1918. Encephalitis Lethargica was a devastating illness that swept the world in the 1920's. Between 1917 to 1928, an epidemic of encephalitis lethargica spread throughout the world, but no recurrence of the epidemic has since been reported. Few studies have been carried out on the encephalitis lethargica epidemic … The Demise of Poskanzer and Schwab's Influenza Theory on the Pathogenesis of Parkinson's Disease. The cause remains a mystery, yet it’s no surprise that the sleepy sickness epidemic is not a well known part of the 1920s history. I. [21] Other sources have suggested Streptococcus pneumoniae as a cause.[22]. [25] Postencephalitic Parkinsonism is perhaps the most widely recognized of such syndromes. [Von Economo-Cruchet lethargic encephalitis and its relation to HIV infection]. The role of the influenza virus in the influenza pandemic of 1918/1919. Some investigators think it may have been seen previously as an outbreak of encephalitis of unknown cause termed (epidemic) encephalitis lethargica that occurred worldwide between 1918 and 1928. He vaccinated patients with early-stage symptoms, telling them that it might be successful. The cases occurred in or in the neighborhood of Kiel, between the end ofNovember, 1918, and April, 1919. [Economo's epidemic encephalitis--unsolved neurologic problem (on the 60th anniversary of the description of Economo's encephalitis by Soviet neuropathologists)]. It attacked the brain, leaving victims like living statues, speechless and motionless. The disease often passes through several stages: fever and delirium, hyposomnia (loss of sleep), and hypersomnia (excessive sleep, sometimes bordering on coma). Unable to load your collection due to an error, Unable to load your delegates due to an error. The epidemic paralleled the 1918 Spanish influenza pandemic and some experts believed both were caused by an influenza virus ( ARNM, 1921 ). a unique encephalitis, presumably viral in origin, which followed the influenza pandemic of 1914-1918. "[26], Modern treatment approaches to encephalitis lethargica include immunomodulating therapies, and treatments to remediate specific symptoms. Clipboard, Search History, and several other advanced features are temporarily unavailable. 1919; 12 (Suppl):1.1–21.1. Please enable it to take advantage of the complete set of features! Comment in J R Soc Med. -, Lancet. They would be conscious and aware – yet not fully awake; they would sit motionless and speechless all day in their chairs, totally lacking energy, impetus, initiative, motive, appetite, affect or desire; they registered what went on about them without active attention, and with profound indifference. 1982 Oct 16;2(8303):860-4. doi: 10.1016/s0140-6736(82)90820-0. [30][31], Atypical form of encephalitis (sleeping sickness), Please review the contents of the article and. [citation needed] Patients may also experience abnormal eye movements ("oculogyric crises"),[13] Parkinsonism, upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including psychosis. A disaster of epic proportions, recent estimates have put the flu’s death toll at 60-100 million worldwide. [15], Some studies have explored its origins in an autoimmune response,[5] and, separately or in relation to an immune response, links to pathologies of infectious disease – viral and bacterial,[5] e.g., in the case of influenza, where a link with encephalitis is clear. The victims of an encephalitis epidemic many years ago have been catatonic ever since, but now a new drug offers the prospect of reviving them. Zh Nevropatol Psikhiatr Im S S Korsakova. -, J Neurol Neurosurg Psychiatry. Encephalitis lethargica epidemic. Stern is author of the definitive 1920s book, Die Epidemische Encephalitis. [28] The disease becomes progressive, with evidence of brain damage similar to Parkinson's disease. About 10 years after von Economo published his description of the disease, the encephalitis lethargica epidemic began to disappear. 1997 Dec;90(12):709-10. [PMC free article] Crookshank FG. One, which has been widely accepted, includes an acute or subacute encephalitic illness where all other known causes of encephalitis have been excluded. 2018 Jun 28;4(1):3. doi: 10.1038/s41572-018-0002-y. COVID-19 and Parkinson's Disease: Are We Dealing with Short-term Impacts or Something Worse? Encephalitis lethargica was a worldwide epidemic during the years 1918-1930 that resembled influenza. Careers. [14], The causes of encephalitis lethargica are uncertain. During the course of the 1917–1926 epidemic, approximately half a million people world-wide were … Dourmashkin RR(1). 1992 Jan-Feb;37(1):46-9 H1N1 virus. In 1918, a world war was raging, and a lethal strain of influenza was circling the globe. By 1918, the number of sporadic cases of encephalitis lethargica reported in Europe and elsewhere increased rapidly andthe conditionbecame epidemic at the sametime 488 h?SS*55a*] ENCEPHALITIS LETHARGICA. Letter from Oliver Sacks to Professor Joel A. Vilensky, 9 Sept 2012. Dourmashkin RR(1). Symptoms included ophthalmoplegia and marked somnolence, and in many survivors, the delayed development of Parkinson disease; the basis for postencephalitic Parkinsonism. A second symptom would lead to an oculogyric crisis. Given that it appears after the acute episode has disappeared, molecular mimetism has been proposed to explain the autoimmune lesion. In late 1916, while treating patients in the Psychiatric-Neurological Clinic of the University of Vienna, Dr Constantin von Economo examined several patients who presented with unusual neurological symptoms. "[11] Hence, while opinions on the relationship of encephalitis lethargica to influenza remain divided, the preponderance of literature appears skeptical. 1981;81(2):124-7. Postencephalitic Parkinson's disease may develop after a bout of encephalitis-sometimes as long as a year after the illness. [24] Until Constantin von Economo identified a unique pattern of damage among the brains of deceased patients and introduced the unifying name encephalitis lethargica, reports of the protean disease came in under a range of names: botulism, toxic ophthalmoplegia, epidemic stupor, epidemic lethargic encephalitis, acute polioencephalitis, Heine-Medin disease, bulbar paralysis, hystero-epilepsy, acute dementia, and sometimes just "an obscure disease with cerebral symptoms. The fast-moving epidemic came in three waves, with the mildest strain erupting in mid-year 1918, targeting the very young and the elderly. Manganese exposure in juvenile C57BL/6 mice increases glial inflammatory responses in the substantia nigra following infection with H1N1 influenza virus. (Liner Notes/ Reference Guide for Abridged Audiobook of Titus Alone), Learn how and when to remove these template messages, Learn how and when to remove this template message, "Encephalitis Lethargica Syndrome: 20 New Cases and Evidence of Basal Ganglia Autoimmunity", "1918 Influenza, Encephalitis Lethargica, Parkinsonism", "The relationship between encephalitis lethargica and influenza: A critical analysis", "Encephalitis lethargica: 100 years after the epidemic", "Experimenting on the Past: The Enigma of von Economo's Encephalitis Lethargica", "The Relationship Between Encephalitis Lethargica and Influenza: A Critical Analysis", "Highly Pathogenic H5N1 Influenza Virus Can Enter the Central Nervous System and Induce Neuroinflammation and Neurodegeneration", Proceedings of the National Academy of Sciences, "Children and Encephalitis Lethargica: A Historical Review", "Evidence for an enterovirus as the cause of encephalitis lethargica", "Encephalitis Lethargica Information Page", "Review: "Asleep: the Forgotten Epidemic That Remains One of Medicine's Greatest Mysteries" [Molly Caldwell Crosby", "Dementia With Lewy Bodies and the Neurobehavioral Decline of Mervyn Peake", National Institute of Neurological Disorders and Stroke, Extranodal NK/T-cell lymphoma, nasal type, Progressive multifocal leukoencephalopathy, https://en.wikipedia.org/w/index.php?title=Encephalitis_lethargica&oldid=1017104804, Viral infections of the central nervous system, Short description is different from Wikidata, Articles needing expert attention from December 2015, Medicine articles needing expert attention, Articles needing additional medical references from December 2015, All articles needing additional references, Articles requiring reliable medical sources, Articles with multiple maintenance issues, Articles with unsourced statements from December 2015, Articles with unsourced statements from October 2020, Wikipedia articles in need of updating from December 2015, All Wikipedia articles in need of updating, Creative Commons Attribution-ShareAlike License, Economo's disease; von Economo's encephalitis, In 1580, Europe was swept by a serious febrile and lethargic illness, which led to, In 1673–1675, a similar serious epidemic occurred in. Eventually known as encephalitis lethargica, or sleeping sickness, it would spread across the world, leaving millions dead or. -. It attacked the brain, leaving victims like living statues, speechless and motionless. An investigation of the characteristics of influenza epidemics in the nineteenth and early twentieth centuries was undertaken, principally in order to analyze the role of the 1918/1919 influenza pandemic in the etiology of encephalitis lethargica. This almost certainly applies to the numerous cases of encephalitis seen after the 1918 epidemic of influenza which culminated in the 1920–21 epidemic of encephalitis lethargica … sporadic encephalitis is known to occur in England and Wales every year whether or not there is an influenza epidemic ( Stuart-Harris 1965 , pp 52f). Synonym (s): encephalitis lethargica, polioencephalitis infectiva What caused the 1918-30 epidemic of encephalitis lethargica? the wound the skin is bright red and swollen. "[19], Subsequent to publication of this compendium, an enterovirus was discovered in encephalitis lethargica cases from the epidemic. [Nov. 2, 1918 the attention of those concerned is attracted by the appearance, of a rash on the body. Then, in 1927, it disappeared as suddenly as it had arrived. There were no encephalitis lethargica … [16] 1963 Jun;90:942-51 J Clin Virol. -, Vopr Virusol. Votes: 122,977 | Gross: $52.10M In 1918, a world war raged, and a lethal strain of influenza circled the globe. Parkinsons Dis. Infection with mosquito-borne alphavirus induces selective loss of dopaminergic neurons, neuroinflammation and widespread protein aggregation. Victorino DB, Guimarães-Marques M, Nejm M, Scorza FA, Scorza CA. Encephalitis lethargica (EL) was a complex and mysterious disease that appeared around the same time as the great influenza pandemic of 1918. Epub 2013 Jun 11. J R Soc Med. Another fascinating foray into medical history from the author of The American Plague. Epub 2014 Jul 30. Stern suspected encephalitis lethargica to be close to poliomyelitis, without evidence. [29][needs update], Treatment is then symptomatic. Encephalitis lethargica was a worldwide epidemic during the years 1918-1930 that resembled influenza. 2014 Oct;61(2):189-95. doi: 10.1016/j.jcv.2014.07.013. Proc R Soc Med. ‘Coma’ was an ambiguous term until the 1930s; it increasingly had the meaning it now has — a state of persistent unconsciousness from which it is impossible to rouse the patient — but in 1918, particularly in Europe, it still sometimes described a state from which it was only extremely difficult to elicit a response. Postencephalitic Parkinson's disease may develop after a bout of encephalitis-sometimes as long as a year after the illness. Bantle CM, Phillips AT, Smeyne RJ, Rocha SM, Olson KE, Tjalkens RB. Pathological studies revealed an encephalitis of the midbrain and basal ganglia, with … An investigation of the characteristics of influenza epidemics in the nineteenth and early twentieth centuries was undertaken, principally in order to analyze the role of the 1918/1919 influenza pandemic in the etiology of encephalitis lethargica. [citation needed], There have been several proposed diagnostic criteria for encephalitis lethargica. In the midst of all this death, a bizarre disease appeared in Europe. Epidemic encephalitis lethargica is produced by viral infections of sleep-wakefulness mechanisms in the hypothalamus, a structure at the upper end of the brainstem. NPJ Parkinsons Dis. Another diagnostic criterion, suggested more recently, says that the diagnosis of encephalitis lethargica "may be considered if the patient’s condition cannot be attributed to any other known neurological condition and that they show the following signs: influenza-like signs; hypersomnolence (hypersomnia), wakeability, ophthalmoplegia (paralysis of the muscles that control the movement of the eye), and psychiatric changes. Also known as "sleeping sickness" or "sleepy sickness" (distinct from tsetse fly-transmitted sleeping sickness), it was first described in 1917 by the neurologist Constantin von Economo[2][3] and the pathologist Jean-René Cruchet.[4]. 2020;10(3):899-902. doi: 10.3233/JPD-202073. [24], The number of people infected during the ten years of the pandemic is unknown, but it is estimated that more than 1 million people contracted the disease, which directly caused more than 500,000 deaths. 2021 Jan 25;16(1):e0245171. Krammer F, Smith GJD, Fouchier RAM, Peiris M, Kedzierska K, Doherty PC, Palese P, Shaw ML, Treanor J, Webster RG, García-Sastre A. Nat Rev Dis Primers. [5][12] In severe cases, patients may enter a coma-like state (akinetic mutism). Encephalitis lethargica, an infectious disorder that only once appeared in epidemic form (1916-1926), is largely forgotten now. The name encephalitis lethargica itself was given by the neurologist Constantin Von Economo in 1916 at an early stage of the epidemic that happened during and after the First World War. Between 1915 and 1926, a world-wide encephalitis lethargica pandemic occurred, impacting nearly 5 million people and killing an estimated 1.6 million people. A Note on the History of Epidemic Encephalomyelitis. 2019 Sep 13;5:20. doi: 10.1038/s41531-019-0090-8. 1981. It was first described in Vienna in 1916 by Constantin von Economo in thirteen patients suffering from unusual neurological symptoms that he thought constituted a new entity and called encephalitis lethargica. Post-encephalitic syndromes varied widely: sometimes they proceeded rapidly, leading to profound disability or death; sometimes very slowly; sometimes they progressed to a certain point and then stayed at this point for years or decades; and sometimes, following their initial onslaught, they remitted and disappeared. Crookshank FG. 2013;2013:167843. doi: 10.1155/2013/167843. Von Economo considered the uniqueness of this set of sy… Proc R Soc Med. Nevertheless, he experimented with the convalescent serum of survivors of the first acute syndrome. In the midst of all this death, a bizarre disease appeared in Europe. A Note on the History of Epidemic Encephalomyelitis. Author information: (1)Department of Medical Microbiology (Academic Virology), St Bartholomew's, London, England. It had a nonspecific prodromal phase, with influenza-like symptoms, and an acute phase, characterised by fever, sleepiness, ocular motility disturbances, and movement disorders. [27], There is little evidence so far of a consistent effective treatment for the initial stages, though some patients given steroids have seen improvement. The Spanish flu epidemic of 1918, the flapper era, the speakeasy and Prohibition, and the Stock Market Crash of 1929 all overshadowed the epidemic of encephalitis lethargica. 1981 Aug;44(8):670-6 1919;12(Suppl):1.1-21.1 eCollection 2019. Lancet. Several cases have been reported through time, but it was with the 1918 flu pandemic, known as the Spanish flu, that lethargic encephalitis reached epidemic levels with cases reported until 1923 in various countries. Contemporary reviews of states of disturbed consciousness: Aschaffenburg 1915; Barker 1917. One theory is that the co-occurrence of the encephalitis and the 1918 influenza pandemic was no coincidence, and that the influenza virus somehow affected the brain in some cases, causing encephalitis lethargica. Bethesda, MD 20894, Copyright The relationship of encephalitis lethargica to the 1918 influenza epidemic is unclear, but we can no longer afford to remain ignorant about it. PLoS One. Difficulty of diagnosis and the lack of statistical records in many countries mean … Accessibility eCollection 2021. Expectations regarding a future influenza pandemic d … Encephalitis lethargica and influenza. 1918 influenza, encephalitis lethargica, parkinsonism. In 1918, influenza caused one of the most severe pandemics in history. Zh Nevropatol Psikhiatr Im S S Korsakova. What caused the 1918-30 epidemic of encephalitis lethargica? [citation needed] Klazomania (a vocal tic) is sometimes present. It was first described in Vienna in 1916 by Constantin von Economo in thirteen patients suffering from unusual neurological symptoms that he thought constituted a new entity and called encephalitis lethargica. AIM: Encephalitis lethargica spread as a pandemic over the period 1918-1921. The first modern plague: epidemic encephalitis in America, 1919-39. [11], Encephalitis lethargica is characterized by high fever, sore throat, headache, lethargy, double vision, delayed physical and mental response, sleep inversion and catatonia. In the fall of 1918, a second wave brought a virulent strain resulting in a seve… Others believe that a virus related to the polio virus was the cause. Muriel "Kit" Richardson (née Hewitt), first wife of actor Sir, Spinal muscular atrophy with lower extremity predominance (SMALED), This page was last edited on 10 April 2021, at 21:03. In reviewing the relationship between influenza and encephalitis lethargica (EL), McCall and coworkers conclude, as of 2008, that "the case against influenza [is] less decisive than currently perceived… there is little direct evidence supporting influenza in the etiology of EL," and that "[a]lmost 100 years after the EL epidemic, its etiology remains enigmatic. In contrast, American Samoa, which is only 32 miles away, maintained a strict quarantine and avoided the 1918 influenza epidemic. Prevention and treatment information (HHS), National Library of Medicine [20] In 2012, Oliver Sacks, the author of the book "Awakenings", about institutionalized EL survivors, acknowledged this virus as the probable cause of the disease. 2002 Dec;24:63-7. The Spanish Influenza pandemic of 1918-19 was the worst pandemic of modern times, claiming over 30 million lives in less than six months. Encephalitis lethargica emerged at around the same time and affected more than one million individuals. PMCID: PMC1296535 PMID: … [7][8][9] Encephalitis lethargica assumed its most virulent form between October 1918 and January 1919. Since the 1916–1927 epidemic, only sporadic cases have been described. [PMC free article] Ravenholt RT, Foege WH. If patients of Stern followed this course of disease, he diagnosed them with encephalitis lethargica. The cause of encephalitis lethargica is unknown. In 1916, von Economo first described encephalitis lethargica (EL), a CNS disorder presenting with pharyngitis followed by sleep disorder, basal ganglia signs (particularly parkinsonism) and neuropsychiatric sequelae. [24] The great encephalitis pandemic coincided with the 1918 influenza pandemic, and it is likely that the influenza virus potentiated the effects of the causative agent of the encephalitis or lowered resistance to it in a catastrophic way. 1919; 12 (Suppl):1.1–21.1. The cause of encephalitis lethargica is unknown. 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