When Bickerstaff’s encephalitis is a disease that is associated with inflammation in the brain stem. In addition, the nerves of the brain are impaired by Bickerstaff encephalitis, so that the patients usually suffer serious disorders of consciousness. Recently, medicine has been investigating the connection between Bickerstaff encephalitis and Miller-Fisher syndrome.
What is Bickerstaff encephalitis?
The first description of Bickerstaff encephalitis was in 1951 by the physician Edwin Bickerstaff. Basically, Bickerstaff encephalitis is a rarely occurring form of encephalitis. It has been suggested that the cause of Bickerstaff encephalitis is found in the body’s own antibodies that are directed against the brain stem. As a result, the brain stem swells significantly.
In some cases, Bickerstaff encephalitis also affects the peripheral nervous system. Initially, patients with Bickerstaff encephalitis experience symptoms that are similar to those of the flu. People feel dull and exhausted, tired and suffer from headaches. In addition, there is usually a more or less severe fever.
In the further course of Bickerstaff’s encephalitis, serious impairments in the function of the brain stem result. The cranial nerves are primarily affected by these disorders. This results in paralysis of the face, double vision and disorders of the swallowing process. In addition, people suffering from Bickerstaff encephalitis suffer from ataxia and impaired consciousness.
The often severe course of Bickerstaff encephalitis usually requires intensive care of the patient. The healing process takes at least a few months, but sometimes takes longer than a year. Nevertheless, the majority of patients with Bickerstaff encephalitis eventually make a complete recovery.
How exactly Bickerstaff encephalitis develops has not yet been researched. However, doctors generally identify a special antiganglioside antibody in patients with Bickerstaff encephalitis, which is also known as ‘anti-GQ1b’. Researchers suspect that Bickerstaff encephalitis enters the organism through an infection of the airways or the gastrointestinal tract.
This presumably creates the causative antibodies. These are not only directed against external pathogens, but also against the body’s own nerve tissue. Because these nerve structures are partly similar to the antigens. Lymphomas and infections with viruses are also possible.
Symptoms, ailments & signs
The symptoms of Bickerstaff encephalitis are expressed in a number of ways. At the beginning of the disease, patients often experience ataxia. The people show motor disorders, show an unsteady gait and generally impaired movements. Ophthalmoplegia also often develops, in which the internal and external muscles of the eyes are paralyzed.
Patients with Bickerstaff encephalitis have double vision or vision that is blurred and out of focus. In addition, the eyelid closure is impaired. In addition, language disorders usually develop. Some patients go into a coma as Bickerstaff encephalitis progresses.
Bickerstaff encephalitis affects long nerve lines that run through the brain stem. As a result, disorders develop in the muscles’ own reflexes. In addition, so-called pyramid orbit signs appear, for example in connection with the Babinski reflex. The symptoms of Bickerstaff encephalitis usually worsen over a period of a week to a month.
Diagnosis & course
The diagnosis of Bickerstaff encephalitis must be made by a specialist, to whom the general practitioner will refer the patient after an initial assessment of the symptoms. The anamnesis primarily asks about the onset of the symptoms of Bickerstaff encephalitis and any previous mild infections.
The clinical examination initially focuses on imaging tests. The doctor usually uses an MRI scan. This is because inflammation in the area of the midbrain, bridge and thalamus can be detected. The doctor also examines the nerve water using laboratory analyzes. Slightly increased concentrations of inflammatory cells and ganglioside antibodies can be detected.
However, a reliable diagnosis of Bickerstaff encephalitis is difficult. Because the Bickerstaff encephalitis shows strong similarities to the Guillain-Barré syndrome and the Miller-Fisher syndrome. Therefore, these two syndromes receive special attention in the differential diagnosis.
Because Bickerstaff’s encephalitis causes inflammation in the brain stem and also affects the nervous functions of the brain, most patients suffer from serious impaired consciousness. Further complications arise when the peripheral nervous system is involved. Symptoms seem harmless at first as they are similar to those of the flu.
Those affected feel tired and exhausted, complain of headaches and a high fever. As the disease progresses, the brain stem and cranial nerves are seriously impaired. These complications result in disturbances in the swallowing process and paralysis of the face.
The patient sees double vision, experiences impairments of consciousness as well as problems with coordination of movements and speaking. Since the long nerve lines in the brain stem are involved, disturbed reflexes of the muscles develop. Other potential side effects are paralysis of the inner and outer eye muscles and impaired eyelid closure. Patients with Bickerstaff encephalitis usually notice the first symptoms within four weeks.
In the case of a severe course of the disease, the clear symptoms can appear after a week. If Bickerstaff encephalitis is detected in good time, the prognosis is comparatively positive. Drug therapies with immunoglobulins and active ingredients from the group of corticosteroids can alleviate or even eliminate symptoms and complaints.
For some patients, it is necessary to relearn individual movements as part of rehabilitation therapy. If left untreated, Bickerstaff encephalitis is fatal.
When should you go to the doctor?
Bickerstaff encephalitis is a rarely occurring form of encephalitis in which the brain stem swells a lot and the cranial nerves are affected. Bickerstaff encephalitis must therefore be treated by a doctor, since otherwise there is a risk of long-term damage to the brain and, in the worst case, death. Under no circumstances should the symptoms be treated with home remedies or over-the-counter medication alone.
Bickerstaff encephalitis is usually noticeable as a disorder of movement coordination. Many patients initially show an unsteady gait. A short time later, other impairments of the motor skills set in, in particular difficulties in writing or grasping. People who notice such signs should see a doctor immediately.
Although these symptoms are not yet a sufficient indication of Bickerstaff encephalitis, they should still be clarified. Symptoms of this kind are rarely harmless, so a visit to a doctor is strongly recommended in any case. This also applies if these symptoms are only noticed temporarily and no reason for this, such as excessive alcohol consumption, can be identified.
Treatment & Therapy
Bickerstaff encephalitis is relatively rare, so that nowadays hardly any effective treatment options have been tried. In addition, there are no corresponding studies that compare the success of various therapeutic measures with regard to Bickerstaff encephalitis. Because of the parallels that Bickerstaff encephalitis has to other diseases with ganglioside antibodies, patients often receive drug therapy with immunoglobulins.
In addition, a so-called plasmapheresis is used. In individual cases, this shows positive effects on the course of Bickerstaff encephalitis. Other medicinal substances from the corticosteroid group are also used to treat Bickerstaff encephalitis. The doctor usually gives the immunoglobulins by means of intravenous injections to increase their effectiveness.
The prognosis of Bickerstaff encephalitis is comparatively positive with timely and adequate drug treatment. However, some patients require that they relearn specific movements as part of rehabilitation.
Outlook & forecast
Although Bickerstaff encephalitis is a serious condition, it can usually be treated very well. In about 50 percent of the cases, the encephalitis heals completely after the therapy. In the other patients, residual symptoms remain, which depend on the extent of the previous damage to the cranial nerves.
As already mentioned, the immune system attacks the body’s own proteins in the nervous system, so that cranial nerves are destroyed. The autoimmune processes are stopped through the use of immunoglobulins and corticosteroids. The nerves in the brain stem can recover. Possible residual symptoms are based on the destruction of structures that are difficult to rebuild.
Rehabilitation begins after immunotherapy. In this phase, some patients have to relearn lost motion sequences. However, the prognosis is usually good for these patients too. However, the length of the rehabilitation phase also depends on the extent of the damage. It can drag on for months or even years. As a rule, however, comparatively minor residual symptoms such as slight movement restrictions or slight swallowing difficulties remain even after severe courses.
However, without treatment, the prognosis is very poor. The disease continues to advance. It does not heal on its own. Therefore, if left untreated, Bickerstaff encephalitis always leads to death.
Targeted prevention of Bickerstaff encephalitis is relatively difficult. Bickerstaff encephalitis usually begins with a mild infection as a result of the pathogen entering the body. However, apart from compliance with normal hygienic standards, there is hardly any possibility of influencing this infection. In the case of clear symptoms of Bickerstaff encephalitis, a suitable doctor should be consulted immediately so that intensive medical treatment of Bickerstaff encephalitis is carried out in good time.
Follow-up care is not possible in most cases of Bickerstaff encephalitis. The disease cannot always be completely cured, so that the person affected suffers from severe disorders of consciousness and thus from significant restrictions in everyday life. Since the disease itself is usually treated with the help of medication, it is important to ensure that it is taken regularly.
This is the only way to improve. Injections can also be given. Interactions with other drugs may need to be discussed with your doctor. Furthermore, many sufferers of Bickerstaff encephalitis have to relearn some everyday movements.
Physiotherapy measures are available to the patient, in which the mobility of the body and extremities is increased again. Some exercises can also be done at home with the help of family or friends. In the case of swallowing difficulties, the person concerned may be dependent on artificial nutrition or on the help of other people in their everyday life.
In general, loving care has a very positive effect on the course of the disease. Since Bickerstaff encephalitis can also lead to psychological complaints or depression, a visit to a psychologist is also helpful. Contact with other people affected can also have a positive effect on the disease.
You can do that yourself
A doctor should be consulted at the first impairment or disturbance. Unsteady gait, double vision or speech disorders are alarming signs that should be investigated as soon as possible.
Self-help with this illness begins with breaking off daily business activities and an immediate visit to the doctor. No medication should be taken until consultation with the doctor has taken place. The independent ingestion of medicines can cause serious complications and acutely worsen the state of health. Inflammation of the cranial nerves cannot be stopped adequately by the person concerned with their own means.
In cooperation with medical care, support for the body’s own defense system is recommended. In order for the organism to have sufficient capacity to minimize inflammation or prevent it from progressing, a stable immune system is necessary. This can be achieved with a healthy and balanced diet, restful sleep and an adequate supply of oxygen. Avoiding harmful substances such as nicotine, alcohol or drugs is essential. They weaken the body and allow the pathogens to spread and multiply further.
Scientists found that mental strength should also be strengthened. A fundamentally positive attitude towards life and a stable psyche have a positive influence on the course of the disease and prevent some possible complications.