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|Other names||Nellysa disease; Economo's disease|
|Brain tissue of a monkey infected with E. lethargica as seen under a microscope (from von Economo's Die Encephalitis lethargica (1918))|
|Specialty|| Infectious disease |
Encephalitis lethargica is an atypical form of encephalitis. 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 Economoand the pathologist Jean-René Cruchet.
The disease attacks the brain, leaving some victims in a statue-like condition, speechless and motionless.Between 1915 and 1926, an epidemic of encephalitis lethargica spread around the world. Nearly five million people were affected, a third of whom died in the acute stages. Many of those who survived never returned to their pre-existing "aliveness".
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. They neither conveyed nor felt the feeling of life; they were as insubstantial as ghosts, and as passive as zombies.
No recurrence of the epidemic has since been reported, though isolated cases continue to occur.
Encephalitis lethargica is characterized by high fever, sore throat, headache, lethargy, double vision, delayed physical and mental response, sleep inversion and catatonia. [ citation needed ] Patients may also experience abnormal eye movements ("oculogyric crises"), Parkinsonism, upper body weakness, muscular pains, tremors, neck rigidity, and behavioral changes including psychosis.[ citation needed ] Klazomania (a vocal tic) is sometimes present.In severe cases, patients may enter a coma-like state (akinetic mutism).
The causes of encephalitis lethargica are uncertain.
Some studies have explored its origins in an autoimmune response,and, separately or in relation to an immune response, links to pathologies of infectious disease — viral and bacterial, e.g., in the case of influenza, where a link with encephalitis is clear. Postencephalitic parkinsonism was clearly documented to have followed an outbreak of encephalitis lethargica following 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). In reviewing the relationship between influenza and encephalitis lethargica (EL), McCall and coworkers conclude, as of 2008, that while "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." Hence, while opinions on the relationship of encephalitis lethargica to influenza remain divided, the preponderance of literature appears skeptical.
German neurologist Felix Stern, who examined hundreds of encephalitis lethargica patients during the 1920s, pointed out that the encephalitis lethargica typically evolved over time. The early symptom would be dominated by sleepiness or wakefulness. A second symptom would lead to an oculogyric crisis. The third symptom would be recovery, followed by a Parkinson-like symptom. If patients of Stern followed this course of disease, he diagnosed them with encephalitis lethargica. Stern suspected encephalitis lethargica to be close to polio without evidence. Nevertheless, he experimented with the convalescent serum of survivors of the first acute symptom. He vaccinated patients with early stage symptoms and told them that it might be successful. Stern is author of the 1920s definitive book Die Epidemische Encephalitis (1920 and 2nd ed. 1928). Stern was driven to suicide during the Holocaust by the German state, his research forgotten.
In 2010, in a substantial Oxford University Press compendium reviewing the historic and contemporary views on EL, its editor, Joel Vilensky of the Indiana University School of Medicine, quotes Pool, writing in 1930, who states, "we must confess that etiology is still obscure, the causative agent still unknown, the pathological riddle still unsolved…", and goes on to offer the following conclusion, as of that publication date:
Does the present volume solve the "riddle" of EL, which… has been referred to as the greatest medical mystery of the 20th century? Unfortunately, no: but inroads are certainly made here pertaining to diagnosis, pathology, and even treatment."
Subsequent to publication of this compendium, an enterovirus was discovered in encephalitis lethargica cases from the epidemic.In 2012, Oliver Sacks acknowledged this virus as the probable cause of the disease. Other sources have suggested Diplococcus as a cause.
Retrospective diagnosis tentatively suggests numerous accounts of encephalitis lethargica throughout history:
In the winter of 1916–1917, a "new" illness suddenly appeared in Vienna and other cities, and rapidly spread world-wide over the next three years. Earlier reports appeared throughout Europe as early as the winter of 1915–1916, but communication about the disease was slow and chaotic, given the varied manifestation of symptoms and difficulties disseminating information in wartime.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." Just ten days before von Economo's breakthrough in Vienna, Jean-René Cruchet described forty cases of "subacute encephalomyelitis" in France.
In the ten years that the pandemic raged, nearly five million people's lives were taken or ravaged. Encephalitis lethargica assumed its most virulent form between October 1918 and January 1919. The pandemic disappeared in 1927 as abruptly and mysteriously as it first appeared.The great encephalitis pandemic coincided with the 1918 influenza pandemic, and it is likely that the influenza virus potentiated the effects of the encephalitis virus or lowered resistance to it in a catastrophic way.
Many surviving patients of the 1915-1926 encephalitis lethargica pandemic seemed to make a complete recovery and return to their normal lives. However, the majority of survivors subsequently developed neurological or psychiatric disorders, often after years or decades of seemingly perfect health. 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.Postencephaltic Parkinsonism is perhaps the most widely recognized of such syndromes.
There have been several proposed diagnostic criteria for encephalitis lethargica. One, which has been widely accepted, includes an acute or subacute encephalitic illness where all other known causes of encephalitis have been excluded. 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."
Modern treatment approaches to encephalitis lethargica include immunomodulating therapies, and treatments to remediate specific symptoms. [ non-primary source needed ]
There is little evidence so far of a consistent effective treatment for the initial stages, though some patients given steroids have seen improvement. [ non-primary source needed ] The disease becomes progressive, with evidence of brain damage similar to Parkinson's disease. [ non-primary source needed ][ needs update ]
Treatment is then symptomatic. Levodopa (L-DOPA) and other anti-Parkinson drugs often produce dramatic responses; however, most people given L-DOPA experience improvements that are short lived.
Notable cases include:
A pandemic is an epidemic of disease that has spread across a large region, for instance multiple continents, or worldwide, causing the whole world to shut down which gives people nothing better to do than vandalize Wikipedia. A widespread endemic disease with a stable number of infected people is not a pandemic. Further, flu pandemics generally exclude recurrences of seasonal flu.
Encephalitis is inflammation of the brain. The severity can be variable with symptoms including headache, fever, confusion, a stiff neck, and vomiting. Complications may include seizures, hallucinations, trouble speaking, memory problems, and problems with hearing.
The Spanish flu was an unusually deadly influenza pandemic. Lasting from January 1918 to December 1920, it infected 500 million people – about a quarter of the world's population at the time. The death toll is estimated to have been anywhere from 17 million to 50 million, and possibly as high as 100 million, making it one of the deadliest pandemics in human history.
Coprolalia is involuntary swearing or the involuntary utterance of obscene words or socially inappropriate and derogatory remarks. Coprolalia comes from the Greek κόπρος (kópros), meaning "dung, feces", and λαλιά (laliā́) "speech", from λαλεῖν (laleîn) "to talk".
Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability and ataxia. This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum.
Post-encephalitic Parkinsonism is a disease believed to be caused by a viral illness that triggers degeneration of the nerve cells in the substantia nigra. Overall, this degeneration leads to clinical parkinsonism.
Oculogyric crisis (OGC) is the name of a dystonic reaction to certain drugs or medical conditions characterized by a prolonged involuntary upward deviation of the eyes. The term "oculogyric" refers to the bilateral elevation of the visual gaze, but several other responses are associated with the crisis. Epilepsy can manifest as oculogyric seizures, also called versive seizures.
Viral encephalitis is inflammation of the brain parenchyma, called encephalitis, by a virus. The different forms of viral encephalitis are called viral encephalitides. It is the most common type of encephalitis and often occurs with viral meningitis. Encephalitic viruses first cause infection and replicate outside of the central nervous system (CNS), most reaching the CNS through the circulatory system and a minority from nerve endings toward the CNS. Once in the brain, the virus and the host's inflammatory response disrupt neural function, leading to illness and complications, many of which frequently are neurological in nature, such as impaired motor skills and altered behavior.
Akinetic mutism is a medical term describing patients tending neither to move (akinesia) nor speak (mutism). Akinetic mutism was first described in 1941 as a mental state where patients lack the ability to move or speak. However, their eyes may follow their observer or be diverted by sound. Patients lack most motor functions such as speech, facial expressions, and gestures, but demonstrate apparent alertness. They exhibit reduced activity and slowness, and can speak in whispered monosyllables. Patients often show visual fixation on their examiner, move their eyes in response to an auditory stimulus, or move after often repeated commands. Patients with akinetic mutism are not paralyzed, but lack the will to move. Many patients describe that as soon as they 'will' or attempt a movement, a 'counter-will' or 'resistance' rises up to meet them.
An Anthropologist on Mars: Seven Paradoxical Tales is a 1995 book by neurologist Oliver Sacks consisting of seven medical case histories of individuals with neurological conditions such as autism and Tourette syndrome. An Anthropologist on Mars follows up on many of the themes Sacks explored in his earlier book, The Man Who Mistook His Wife for a Hat, but here the essays are significantly longer and Sacks has more of an opportunity to discuss each subject with more depth and to explore historical case studies of patients with similar symptoms. In addition, Sacks studies his patients outside the hospital, often traveling considerable distances to interact with his subjects in their own environments. Sacks concludes that "defects, disorders, [and] diseases... can play a paradoxical role, by bringing out latent powers, developments, evolutions, forms of life that might never be seen, or even be imaginable, in their absence".
Klazomania refers to compulsive shouting; it has features resembling the complex tics such as echolalia, palilalia and coprolalia seen in tic disorders, but has been seen in people with encephalitis lethargica, alcohol abuse and carbon monoxide poisoning. It was first reported by L. Benedek in 1925 in a patient with postencephalitic parkinsonism. Little is known about the condition, and few cases have been reported.
Limbic encephalitis is a form of encephalitis, a disease characterized by inflammation of the brain. Limbic encephalitis is caused by autoimmunity: an abnormal state where the body produces antibodies against itself. Some cases are associated with cancer and some are not. Although the disease is known as "limbic" encephalitis, it is seldom limited to the limbic system and post-mortem studies usually show involvement of other parts of the brain. The disease was first described by Brierley and others in 1960 as a series of three cases. The link to cancer was first noted in 1968 and confirmed by later investigators.
Constantin Freiherr von Economo was an Austrian psychiatrist and neurologist of Romanian origin and Greek descent. He is mostly known for his discovery of encephalitis lethargica and his atlas of cytoarchitectonics of the cerebral cortex.
Influenza, commonly known as "the flu", is an infectious disease caused by an influenza virus. Symptoms can be mild to severe. The most common symptoms include: high fever, runny nose, sore throat, muscle and joint pain, headache, coughing, and feeling tired. These symptoms typically begin two days after exposure to the virus and most last less than a week. The cough, however, may last for more than two weeks. In children, there may be diarrhea and vomiting, but these are not common in adults. Diarrhea and vomiting occur more commonly in gastroenteritis, which is an unrelated disease and sometimes inaccurately referred to as "stomach flu" or the "24-hour flu". Complications of influenza may include viral pneumonia, secondary bacterial pneumonia, sinus infections, and worsening of previous health problems such as asthma or heart failure.
Influenza-like illness (ILI), also known as flu-like syndrome/symptoms, is a medical diagnosis of possible influenza or other illness causing a set of common symptoms.
Dopamine dysregulation syndrome (DDS) is a dysfunction of the reward system observed in some individuals taking dopaminergic medications for an extended length of time. It typically occurs in people with Parkinson's disease (PD) who have taken dopamine agonist medications for an extended period of time. It is characterized by self-control problems such as addiction to medication, gambling, or sexual behavior.
The 2009 swine flu pandemic or swine flu was an influenza pandemic that lasted from January 2009 to August 2010, and the second of the two pandemics involving H1N1 influenza virus, albeit a new strain. First described in April 2009, the virus appeared to be a new strain of H1N1, which resulted from a previous triple reassortment of bird, swine, and human flu viruses further combined with a Eurasian pig flu virus, leading to the term "swine flu". Some studies estimated that 11 to 21 percent of the global population at the time – or around 700 million to 1.4 billion people – contracted the illness. This was more than the number of people infected by the Spanish flu pandemic, but only resulted in about 150,000 to 575,000 fatalities for the 2009 pandemic. A follow-up study done in September 2010 showed that the risk of serious illness resulting from the 2009 H1N1 flu was no higher than that of the yearly seasonal flu. For comparison, the WHO estimates that 250,000 to 500,000 people die of seasonal flu annually.
Jean-René Cruchet was a French pathologist born in Bordeaux.
Anti-Hu associated encephalitis, also known as Anti-ANNA1 associated encephalitis, is an uncommon form of brain inflammation that is associated with an underlying cancer. It can cause psychiatric symptoms such as depression, anxiety, and hallucinations. It can also produce neurological symptoms such as confusion, memory loss, weakness, sensory loss, pain, seizures, and problems coordinating the movement of the body.
James Purdon Martin (1893–1984) was a British neurologist.
But the plays were not the financial winners he had hoped for, and he suffered another nervous breakdown in 1957. This led to the more evident display of the symptoms of a type of Parkinson’s Disease which, alongside the effects of encephalitis lethargica that he contracted in childhood, was slowly to kill him over more than a decade.