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Encephalitis lethargica is an atypical form of encephalitis.
Encephalitis Lethargica, also known as sleeping sickness (though different from the sleeping sickness transmitted by the tsetse fly), is a devastating illness that swept the world in the 1920s and then vanished as quickly as it had appeared. Encephalitis Lethargica attacks the brain, leaving some victims like living statues, speechless and motionless. It is as if the victims go to sleep and do not wake up.
Between 1917 and 1928, an epidemic of encephalitis lethargica spread throughout the world, but no recurrence of the epidemic has since been reported, though isolated cases continue to occur. During the outbreak, over a million died, and some were left frozen inside their useless bodies, in institutions.
Symptoms
It is characterized by high fever, sore throat, headache, double vision, delayed physical and mental response, sleep inversion, catatonia and lethargy. In acute cases, patients may enter coma. 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 illness.
Cause
The cause of encephalitis lethargica is not yet known for certain, but on the basis of research by British doctors Russell Dale and Andrew Church, the disease is now thought to be due to a massive immune reaction to an infection by the streptococcus-like bacterium, diplococcus. There is also some evidence of an autoimmune origin with antibodies (IgG) from patients with EL binding to neurones in the basal ganglia and mid-brain. It had been hypothesised that encephalitis lethargica, Sydenham's chorea and PANDAS are mediated by the same post-streptococcal immune response.
Treatment
Treatment for encephalitis lethargica in the early stages is patient stabilisation, which may be very difficult. There is little evidence so far of a consistent effective treatment for the initial stages, though one patient who was given steroids initially has so far made a good recovery. 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 antiparkinson 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 levodopa could produce some amelioration of the symptoms was described in the book Awakenings by Oliver Sacks in 1973, which was made into a film starring Robin Williams and Robert De Niro in 1990. 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.
The encephalitis lethargica epidemic of 1917-1928 has also appeared in comic book literature in Neil Gaiman's Sandman graphic novels, where in the very first story, in 1987, he ascribes the "sleeping sickness" to Morpheus' imprisonment. Finally, progressive metal band Dream Theater appear to use the story to form the basis of the second part of the title track of their 2005 album Octavarium.
The song L Dopa by Big Black is about a woman named Daisy, whose "sleeping sickness" is cured by usage of the drug used in the title.
In the Canadian television show Regenesis, a weaponized strain of Encephalitis lethargica was thought to be the cause of sickness to a number of American soldiers, during the last three episodes of the second season.
References
- 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
- BBC health article on encephalitis lethargica
- NIH.gov
"Encephalitis lethargica"