Encephalitis lethargica
From Wikipedia, the free encyclopedia
| 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,[1][2] 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.[5][6]
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[edit] Symptoms
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 ("oculogyric crises"),[7] parkinsonism, upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including psychosis.
Postencephalitic Parkinsons' disease may develop after a bout of encephalitis, sometimes as long as a year after the start of the illness.
[edit] Cause
The cause of encephalitis lethargica is not known for certain.[8]
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. Western immunoblotting showed that 95% of EL patients had autoantibodies reactive against human basal ganglia antigens.By contrast, antibodies reactive against the basal ganglia were found in only 2-4% of child and adult controls (n = 173, P < 0.0001).[3][citation needed]
Some researchers believe that new data supports the influenza hypothesis,[9] while others consider this less likely.[3]
[edit] 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.[10] 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.
[edit] 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 and Robert De Niro based on the book.
The disease is researched and mentioned in the Canadian television show ReGenesis, in the last few episodes of the second series.
[edit] 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 d e 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:. PMID 14570817. http://brain.oxfordjournals.org/cgi/content/full/127/1/21.
- ^ 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.
- ^ Vilensky JA, Goetz CG, Gilman S (January 2006). "Movement disorders associated with encephalitis lethargica: a video compilation". Mov. Disord. 21 (1): 1–8. doi:. PMID 16200538.
- ^ McCall S, Vilensky JA, Gilman S, Taubenberger JK (May 2008). "The relationship between encephalitis lethargica and influenza: a critical analysis". J. Neurovirol. 14 (3): 177–85. doi:. PMID 18569452. http://www.informaworld.com/openurl?genre=article&doi=10.1080/13550280801995445&magic=pubmed||1B69BA326FFE69C3F0A8F227DF8201D0.
- ^ Vilensky JA, Foley P, Gilman S (August 2007). "Children and encephalitis lethargica: a historical review". Pediatr. Neurol. 37 (2): 79–84. doi:. PMID 17675021. http://linkinghub.elsevier.com/retrieve/pii/S0887-8994(07)00194-4.
- ^ 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:. PMID 9159730.
It is also thought by some that encephalitis lethargica is the explanation for the symptoms of the afflicted in New England during the 1600s, which ultimately resulted in the Salem Witch Trials. The symptoms are consistent, and indeed "a similar epidemic with nearly exact symptoms (to those exhibited in Salem) swept the world from 1916 to 1930. This world-wide pandemic...encephalitis..."
[edit] 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
