Bickerstaff Encephalitis: Causes, Symptoms & Treatment

Bickerstaff encephalitis is a disease associated with inflammation in the brainstem. In addition, the nerves of the brain are affected by Bickerstaff encephalitis, so patients usually suffer severe disorders of consciousness. Recently, the medical community has been increasingly investigating the link between Bickerstaff encephalitis and Miller-Fisher syndrome.

What is Bickerstaff encephalitis?

Bickerstaff encephalitis was first described in 1951 by Edwin Bickerstaff, a physician. Basically, Bickerstaff encephalitis represents a rarely occurring manifestation of encephalitis. It is believed that the cause of Bickerstaff encephalitis is found in the body’s own antibodies directed against the brain stem. As a result, the brainstem swells significantly. In some cases, Bickerstaff encephalitis also affects the peripheral nervous system. Initially, patients suffering from Bickerstaff encephalitis experience symptoms similar to those of the flu. Thus, the persons feel dull and weary, are tired and suffer from headaches. In addition, there is usually a more or less severe fever. As Bickerstaff encephalitis progresses, serious impairments in brainstem function result. These impairments primarily affect the cranial nerves. This results in paralysis of the face, seeing double images, and disturbances in the swallowing process. In addition, people suffering from Bickerstaff encephalitis experience ataxias and impaired consciousness. The often severe course of Bickerstaff encephalitis usually requires intensive patient care. The healing process extends over at least several months, but in some cases lasts longer than a year. Nevertheless, the majority of patients with Bickerstaff encephalitis eventually make a complete recovery.

Causes

Exactly how Bickerstaff encephalitis develops is not currently understood. Basically, however, doctors identify a specific antiganglioside antibody, also known as ‘anti-GQ1b’, in patients with Bickerstaff encephalitis. Researchers suspect that Bickerstaff encephalitis enters the body through an infection of the airways or gastrointestinal tract. This presumably gives rise to the causative antibodies. These are directed not only against external pathogens, but also against the body’s own tissue of the nerves. This is because these nerve structures resemble the antigens to some extent. However, lymphomas as well as infections with viruses are also possible.

Symptoms, complaints, and signs

Symptoms of Bickerstaff encephalitis manifest in several ways. At the beginning of the disease, patients often experience ataxia. In this, individuals exhibit disturbances in motor function, show unsteady gait and generally impaired movements. Ophthalmoplegia also often develops, with paralysis of the inner and outer muscles of the eyes. Patients with Bickerstaff encephalitis see double vision or blurred and out of focus. In addition, eyelid closure is impaired. In addition, speech disorders usually develop. Some patients go into a coma as Bickerstaff encephalitis progresses. Bickerstaff encephalitis affects long nerve conduits that run through the brainstem. As a result, disturbances develop in the intrinsic reflexes of the muscles. In addition, so-called pyramidal tract signs appear, for example in connection with the Babinski reflex. In most cases, the symptoms of Bickerstaff encephalitis intensify over a period of one week to one month.

Diagnosis and course

It is essential that the diagnosis of Bickerstaff encephalitis be made by a specialist to whom the general practitioner refers the patient after an initial assessment of the symptoms. The history primarily asks about the onset of symptoms of Bickerstaff encephalitis and any previous mild infections. The clinical examination initially focuses on imaging techniques. In doing so, the physician usually uses an MRI examination. This is because inflammation in the area of the midbrain, bridge and thalamus can be detected. The physician also examines the neural fluid by means of laboratory analyses. Slightly increased concentrations of inflammatory cells and ganglioside antibodies can be detected. However, a reliable diagnosis of Bickerstaff encephalitis is difficult to make.This is because Bickerstaff encephalitis has strong similarities to Guillain-Barré syndrome as well as Miller-Fisher syndrome. Therefore, these two syndromes receive special attention in the differential diagnosis.

Complications

Because Bickerstaff encephalitis causes inflammation in the brainstem and also affects the neural functions of the brain, most patients suffer from severe disorders of consciousness. Further complications present themselves when the peripheral nervous system is involved. Symptoms appear harmless at first, resembling those of influenza. Sufferers feel tired and listless, complain of headaches and high fever. As the disease progresses, serious impairments of brainstem function and cranial nerves develop. These complications result in disturbances in the swallowing process and paralysis of the face. The patient sees double images, experiences impaired consciousness, and problems with movement coordination and speech. Because the long nerve conductors in the brainstem are involved, impaired intrinsic reflexes of the muscles develop. Other potential associated symptoms include paralysis of the internal and external eye muscles and impaired eyelid closure. Patients with Bickerstaff encephalitis usually notice the first symptoms within four weeks. In severe cases, definite symptoms may appear as early as one week. If Bickerstaff encephalitis is detected in time, the prognosis is comparatively positive. Drug therapies with immunoglobulins and agents from the group of corticosteroids can alleviate or completely eliminate the symptoms and complaints. In some patients, it is necessary to relearn individual movements as part of rehabilitation therapy. Without treatment, Bickerstaff encephalitis is fatal.

When should you see a doctor?

Bickerstaff encephalitis is a rare form of brain inflammation that causes severe swelling of the brainstem and affects the cranial nerves. Bickerstaff encephalitis must therefore be treated by a doctor, 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 medications alone. Bickerstaff encephalitis is usually manifested by a disturbance in the coordination of movement. Many patients initially exhibit an unsteady gait. Shortly thereafter, other motor function impairments, especially difficulties with writing or grasping, become apparent. People who notice such signs in themselves should consult a doctor immediately. Although these symptoms are not yet a sufficient indication of Bickerstaff encephalitis, they should nevertheless be clarified. Complaints of this kind are rarely harmless, so that a visit to the doctor is urgently recommended in any case. This is true even if these symptoms are noticed only temporarily and no reason, such as excessive alcohol consumption, is apparent for this.

Treatment and therapy

Bickerstaff encephalitis occurs relatively rarely, so effective treatment options have hardly been tested at present. In addition, there is a lack of appropriate studies comparing the success of different therapeutic interventions with respect to Bickerstaff encephalitis. Because of the parallels between Bickerstaff encephalitis and other diseases with ganglioside antibodies, patients often receive drug therapy with immunoglobulins. In addition, so-called plasmapheresis is used. In individual cases, this has shown positive effects on the course of Bickerstaff encephalitis. Other medical agents from the corticosteroid group are also used to treat Bickerstaff encephalitis. The physician usually administers the immunoglobulins by intravenous injection to increase effectiveness. The prognosis of Bickerstaff encephalitis is comparatively favorable with timely and adequate drug treatment. However, some patients require re-learning of specific movements as part of rehabilitation.

Outlook and prognosis

Although Bickerstaff encephalitis is a serious disease, however, it can usually be treated very well. In about 50 percent of cases, encephalitis heals completely after therapy.In other patients, residual symptoms remain, depending on the extent of previous damage to the cranial nerves. As mentioned above, the immune system attacks the body’s own proteins of the nervous system, resulting in the destruction of cranial nerves. The use of immunoglobulins and corticosteroids stops the autoimmune processes. The nerves of the brain stem can recover. Possible residual symptoms are due to the destruction of structures that are difficult to rebuild. After immunotherapy, the period of rehabilitation begins. Some patients have to relearn lost movements during this phase. Even in these patients, however, the prognosis is usually good. However, the length of the rehabilitation phase also depends on the extent of the damage. It can last for months or even years. As a rule, however, even after severe courses, comparatively minor residual symptoms often remain, such as slight movement restrictions or mild swallowing difficulties. Without treatment, however, the prognosis is very poor. The disease continues to progress. It does not heal on its own. Therefore, without therapy, Bickerstaff encephalitis always leads to death.

Prevention

Targeted prevention of Bickerstaff encephalitis is relatively difficult. Bickerstaff encephalitis usually begins with a mild infection as a result of the entry of the pathogens into the body. However, there is hardly any possibility to influence this infection except observance of normal hygienic standards. In case of clear symptoms of Bickerstaff encephalitis, an appropriate physician should be consulted immediately, so that intensive medical therapy of Bickerstaff encephalitis is provided in time.

Follow-up

Follow-up care is not possible in most cases of Bickerstaff encephalitis. The disease cannot always be completely cured, so the affected person suffers from severe disturbances of consciousness and thus significant limitations in everyday life. Since the disease itself is usually treated with the help of medications, care must be taken to ensure that these are taken regularly. This is the only way to achieve an improvement. Injections can also be administered. It may be necessary to discuss interactions with other medications with the doctor. Furthermore, many patients affected by Bickerstaff encephalitis have to relearn some everyday movements. For this purpose, physiotherapy measures are available to the patient, in which the mobility of the body and the extremities is increased again. Some exercises can be done at home with the help of family or friends. In the case of swallowing difficulties, the affected person may be dependent on artificial feeding or on the help of other people in his or her daily 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 affected individuals can also have a positive effect on the disease.

What you can do yourself

A doctor should be consulted at the first signs of impairment or disturbance. Gait unsteadiness, seeing double images, or disturbances in speech are alarming signs that should be followed up as soon as possible. Self-help for this condition begins with a cessation of everyday business activities and an immediate visit to the doctor. No medication should be taken until consultation with the doctor has taken place. Taking medicines on one’s own responsibility can cause serious complications and acutely worsen the state of health. The inflammation of the cranial nerves cannot be stopped sufficiently by the affected person with his own possibilities. In cooperation with medical care, support of 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 sufficient oxygen supply. The avoidance of harmful substances such as nicotine, alcohol or drugs is elementary. They weaken the body and enable pathogens to spread and multiply further. Scientists found that, in addition, mental strength should be strengthened.A basically positive attitude to life and a stable psyche have a positive influence on the course of the disease and prevent some possible complications.