European sleeping sickness is the name given to an inflammation in the brain that can be accompanied by sudden severe loss of consciousness and neurological deficits. Affected individuals fall uncontrollably into a deep sleep and are often unresponsive afterward. Many find themselves in complete physical and mental torpor. Headaches, nausea and fever often follow. The disease occurred more frequently in Europe between 1915 and 1927 and even led to deaths during this period. Later, only very rare outbreaks were recorded.
What is European sleeping sickness?
The disease was discovered and first described in 1916 by the Austrian psychiatrist and neurologist Constantin Freiherr Economo von San Serff (1876-1931). It was named Economo’s disease after him. More familiar is the name Encephalitis lethargica. Sufferers were often surprised by sleep in the most uncomfortable postures, for example, while eating or in the middle of work. Some could be easily awakened, but others suffered rapid death. Economo noted frequent paralysis of the eye muscles and came across case descriptions from previous centuries set on the European continent. Impressive descriptions of the fates of patients suffering from European sleeping sickness were left by the famous British neurologist Oliver Sacks (1933-2015). At the end of the 1960s, as a young doctor in the neurology department of a hospital in the USA, he met some victims of European sleeping sickness, specifically the epidemic in the 1920s. Using a special nerve agent, he was able to restore consciousness to several patients within a short period of time. Some of them had thus awakened from their torpor after years of mental stagnation. Because they could not cope with this completely new personal situation, they fell back into general rigidity or developed other mental illnesses.
Causes
Most often, encephalitis is caused by viruses. Less commonly, bacteria or other pathogens are the triggers of brain inflammation. Children, young adults, and patients with compromised immune systems are particularly at risk. The viruses (for example, herpes simplex viruses, varicella zoster virus, Epstein-Barr virus) either cause inflammation directly in the brain or block the body’s own defense system. Pathogens under consideration also include protozoa, parasites, and fungi.
Symptoms, complaints, and signs
Encephalitis may be indicated by sudden high fever, nausea, unaccustomed severe headache, and initially mild clouding of consciousness. Concentration and memory show abrupt deficiencies. There are marked changes in behavior, including mood swings and disorientation. Speech and language may be slightly impaired. If left untreated, the disease very often leads to death. If it is diagnosed early and treated properly, there is a very good chance of recovery. Only rarely does permanent damage to the nervous system occur. However, they can also occur many years later. Therefore, the neurological department is always responsible for encephalitis in the clinic. Treated with medication, around 80 percent of patients recover. A life-threatening risk is posed by persistent seizures and swelling of the brain.
Diagnosis and course
The physician’s diagnosis of suspected European sleeping sickness is extraordinarily multifaceted. He will need information about general complaints, past medical history, and regarding possible viral infections. In addition, he will listen to descriptions of relatives or companions, because the encephalitis patient often has striking problems with the ability to perceive and communicate. It is also important to obtain information about recent travel and possible contact with persons at risk, such as those suffering from encephalitis. The physician then uses physical and neurological examinations to test the patient’s chest and head area in particular, as well as his reflexes and reactions to stimuli. He can detect and interpret irregularities of the skin as well as impairments of the water balance. If encephalitis or especially encephalitis lethargica is suspected, the physician will thoroughly examine the blood and cerebrospinal fluids to find out the type and dangerousness of the pathogen as well as existing signs of inflammation.However, reliable findings on this can often only be obtained after several weeks. To rule out possible brain diseases elsewhere, the physician may also rely on the evaluation of computed tomography scans, magnetic resonance imaging scans, and electroencephalography scans.
Complications
European sleeping sickness is due to inflammation of the brain. This is now relatively rare, but can result in complications due to the severity of the disease. Encephalitis lethargica or encephalitis vienna can lead to Parkinson’s-like symptoms or conditions resembling a waking coma. Neurological disorders that appear years later may be considered a further complication of this disease. The American neurologist Oliver Sacks was able to temporarily correct such symptoms with the administration of L-dopa. In some of the patients who were brought back to life, this led to tragic complications. They could not tolerate the new state of wakefulness after years of dozing. As a result, they became depressed or mentally ill. The prognosis for such complications of European sleeping sickness is usually poor. The problem is that European sleeping sickness cannot be traced back to a specific pathogen. Due to initially unspecific symptoms, the risks for complications occurring later are relatively high. For example, a persistent seizure (status epilepticus) may occur. In other cases, brain edema develops due to swelling in the brain. Both complications of European sleeping sickness can have life-threatening consequences for those affected. Since the causative agent of European sleeping sickness has not yet been identified, complications can hardly be ruled out. Thus, Parkinson’s syndrome may occur as a consequence of European sleeping sickness.
When should one go to the doctor?
If there is a sudden onset of high fever, unusually intense headache, and other signs of encephalitis, emergency medical attention should be called. Mood swings and changes in behavior are other warning signs that are best clarified immediately. If unconsciousness occurs, emergency medical services should be alerted immediately. Until specialist help arrives, it is advisable to check the pulse and heartbeat regularly. In addition, the victim must be placed in the recovery position to prevent suffocation. Afterwards, he usually has to spend some time in the hospital. Whether further visits to the doctor are necessary depends very much on how the European sleeping sickness progresses. In case of pain, seizures and other complaints, the responsible doctor should be consulted in any case. Children, elderly people and patients with a compromised immune system are particularly likely to contract encephalitis. It is advisable for these risk groups to be specifically examined for encephalitis after a viral infection. Normally, the earlier the disease is detected, the better the chances of cure.
Treatment and therapy
Treatment of encephalitis is always carried out in an inpatient clinic, because potentially life-threatening exacerbations of the course of the disease can be detected and compensated for immediately here. Likewise, medication administration can be tracked without gaps and modified according to the type of pathogen and severity of the disease. In the initial phase of encephalitis, certain drugs may be ineffective as long as there is no definitive clarity about the trigger of the disease. They are then immediately replaced in the hospital with more appropriate agents. The use of medications for fever reduction, against brain changes, pain and possible seizures is likewise precisely tailored to the needs of the patient in the hospital.
Outlook and prognosis
In European sleeping sickness, which occurs only sporadically today, irreversible damage occurs in the neuronal network of the brain, making it impossible to cure the disease. Although the sleep attacks are initially temporary, the disease nevertheless progresses and damages the neuron network more and more. In the acute state, affected patients suddenly fall asleep, often maintaining uncomfortable postures. The sleep is so deep that it often resembles a coma-like state. At the time of the greatest spread of the disease in the years between 1915 and 1927, it was found that about one-third of those affected died. Death often occurs very rapidly, although the sufferers always remain wakeable.Surviving patients later suffer from paralysis of the eye muscles and eyelid paralysis, among other symptoms. Many years after the sleep attacks, neurological problems occur, which in turn lead to the same symptoms as in the initial stage and then end in complete mental derangement of the patient. Those affected appear oblivious and lapse into complete rigidity. The neurologist Oliver Sacks achieved success with an experimental L-Dopa therapy. L-Dopa has a stimulating effect and can bring patients out of the state of rigidity. However, this awakening from complete mental torpor is often short-term. Some patients fall back into mental torpor because they cannot cope with the new situation after awakening.
Prevention
For the prevention of the brain disease encephalitis, vaccinations are recommended, which can help against the most diverse pathogens. Because they fight similar strains of pathogens, vaccinations against measles, mumps, rubella, and polio are equally appropriate for increased resistance to Economo disease. There are also a wide variety of special vaccinations for people at increased risk of neurological disease. In this regard, special attention should be paid to serum administration against early summer meningoencephalitis (TBE), which is transmitted by ticks. TBE is a brain inflammation whose viral pathogens are very active. Some risk areas for this disease, which mainly occurs in spring and summer, also exist in Germany. Travelers to Southeast Asia are also advised a preventive vaccination against the so-called Japanese encephalitis, which is common there.
Aftercare
In most cases, no measures of aftercare are available to those affected by this disease. The disease itself must be examined and treated in the first place directly by a doctor, so that there are no further complications that can continue to complicate the everyday life of the affected person. The affected person should consult a doctor at the first signs of this disease so that it can be treated quickly. If the disease is left untreated, it can lead to serious complications that significantly reduce the quality of life of the affected person. In most cases, patients with this condition are dependent on treatment by a psychologist. In this regard, the treatment should be carried out regularly in order to properly alleviate the symptoms. The affected person should avoid stress in his life in any case, because it promotes the disease in any case. In this regard, various techniques for relaxation can limit the disease, while also making the patient’s life easier. Also the contact to other patients with the same disease can be useful, because it comes to an exchange of information. As a rule, life expectancy is not reduced by this disease.
What you can do yourself
Since encephalitis can always be life-threatening, one of the most important self-help measures is to interpret the symptoms correctly and seek medical attention promptly. The first signs, in addition to fever and nausea, are very severe headaches as well as behavioral changes that manifest themselves in particular in the form of mood swings and disorientation. Such symptoms must by no means be played down as accompanying symptoms of an incipient flu, but must be discussed immediately with a doctor. Children and adolescents, as well as patients with a weak immune system, are particularly at risk. The immune system can be positively influenced by lifestyle. A healthy diet rich in vitamins and fiber on a predominantly vegetable basis, as well as regular exercise in the fresh air, strengthen the body’s defenses. Unhealthy foods, in particular fatty meat, sausage, sugar and white flour products, as well as excessive consumption of alcohol and cigarettes, should be avoided as far as possible. People with an increased risk of neurological disease should actually make use of the special protective vaccinations that are offered for them. In addition, vaccinations against certain childhood diseases such as measles, mumps and rubella can also reduce the risk of contracting European sleeping sickness.
