Being a prisoner of one’s own body – a terrible idea that becomes oppressive truth in locked-in syndrome (in German: Gefangensein-Syndrom or Eingeschlossensein-Syndrom). The best-known, media-present example today is probably Stephen Hawking.
What is locked-in syndrome?
Locked-in syndrome is a complete paralysis of the four limbs and the body, as well as the speech apparatus, which leads to an almost complete loss of the ability of the person to communicate with his environment. The affected person can usually only communicate via eye movements (blinking, blinking, etc.), but even in this way only very limited expressions are possible through yes/no questions (or and/or questions). If this possibility of communication is lost, help can only be given by technical means in order to maintain an active contact to the outside world. However, it should be noted umbedingt that this condition is by no means a wakeful comatose state, since the patient has his or her full consciousness, i.e., can hear, see, and understand his or her environment.
Causes
The most common cause of this paralytic disorder is brainstem infarction. In this case, the blood supply to the midbrain, cerebral bridge, and medulla oblongata is so severely reduced or, in some cases, completely interrupted that there are significant limitations in various bodily functions. Other common causes are meningitis (meningitis), specific nerve diseases (e.g., amyotrophic lateral sclerosis), strokes, and severe trauma and accidents. More rarely, locked-in syndrome can be observed in patients with multiple sclerosis, arteritis/nerve inflammation, or after abuse of toxic substances/drugs (heroin).
Symptoms, complaints, and signs
Locked-in syndrome is associated with an intact state of consciousness with almost complete inability to act. Affected individuals perceive stimuli. Thus, they can hear, smell, taste, see, and also feel (to a limited extent). Speech comprehension is usually not impaired. The paralyses that occur in locked-in syndrome include the four extremities and horizontal eye movements. In most cases, the ability to speak, swallow and make facial expressions is lost. Thus, only vertical eye movements remain for communication. If these fail, at least the mechanisms for dilating the pupils are still intact. Overall, the physical situation from the neck down can be compared to the situation of completely paraplegic patients. The affected persons are not limited in their wakefulness. In the broadest sense, they experience a normal biorhythm. There is hardly any perceived pain or uncomfortable body sensation. Awareness of their own paralysis is present. The cognitive possibilities are mostly limited only insofar as the trigger of the Locked-In-Syndrome can lead to cognitive limitations. Due to the fact that the patients are usually fully conscious, the locked-in syndrome must be distinguished from the waking coma. In the latter, it must be questioned whether and to what extent those affected are aware of their surroundings.
Diagnosis and course
The diagnosis of LiS cannot be made by purely “visual inspection”, since the clinical picture has a lot of similarity to the vegetative state or akinetic mutism (a disease that is characterized primarily by a severe drive disorder). Suitable diagnostic methods are mainly electrical and magnetic measurements of brain and muscle activity. By means of CT and MRI, changes in blood flow and metabolism of the brain can be detected. These technical diagnostic methods are usually combined with laboratory techniques, for example, to better assess the inflammatory state of meningitis. The course of this disease is very individual and depends both on his medical care and on the cause of the outbreak. Thus, it can be assumed that a mortality of 59-70% occurs when the LiS was caused by a hemorrhage or blockage in brain vessels. In the case of trauma, tumors, etc., this rate drops to about 30%. Diseases triggered by toxins (poisons/drugs) almost never lead to death.
Complications
As a rule, those affected by locked-in syndrome suffer from considerable psychological discomfort and complications.However, they cannot express themselves to the outside world and cannot communicate with it. This leads to clear and considerable restrictions in the everyday life of the affected person. Patients with locked-in syndrome usually suffer from paralysis and are therefore dependent on the help of other people in their daily lives. This often results in movement restrictions, so that the patients are dependent on a wheelchair. Due to the speech disorders, communication with the outside world is usually not possible. The patients themselves are in a waking coma and suffer from severe depression and other psychological upsets. In most cases, the life expectancy of the patient is not limited by the locked-in syndrome. However, the further course depends strongly on the cause of the locked-in syndrome, so that a general course of the disease cannot be predicted. A causal treatment of locked-in syndrome is usually not possible. Those affected are dependent on various therapies and assistance in everyday life. As a rule, the syndrome cannot be completely cured either. Especially the patient’s relatives suffer from significant depression and other psychological limitations due to the syndrome.
When should you go to the doctor?
Locked-in syndrome, by definition, prevents the sufferer from seeing a doctor on his or her own. However, in any case, the worrisome symptomatology leads the sufferer to a hospital. Since a stroke is the most common trigger of locked-in syndrome, medical monitoring usually results after the incident. Sufferers of locked-in syndrome also generally do not have the option of forgoing medical attention. This is because the condition must be urgently differentiated from other states of immobility and appropriate care and attention must be provided. Because the affected person cannot communicate purposefully and the symptomatology of the condition is so easily confused, it is also sometimes incumbent on family members to point out the possibility of locked-in syndrome. Since the condition requires a great deal of medical attention, neurologists are especially important in the further course of the condition to check the functionality of the body. For the course of a possible recovery, it is important that physiotherapeutic, logopedic, occupational therapy and, if necessary, psychotherapeutic treatment is optimally covered by specialists.
Treatment and therapy
Treatment of the affected person requires first and foremost one thing:
An intensive and individualized combination of occupational therapy, speech therapy, and physiotherapy. The main goal here is to mobilize the patient and thus release him from his inability to move. The sooner such rehabilitation is initiated, the more likely it is to be successful. In physiotherapy today, the principle of “systematic repetitive basic training” is primarily applied. This involves initially training only individual, small movements at joints. Once these can be performed independently again and certain positions can be maintained, the training exercises are extended to several joints and muscle groups and later practiced in precise activities (for example, holding a fork and guiding it to the mouth). Further assistance in relearning various skills is provided by occupational therapy, the goals of which are primarily to rebuild fine and gross motor skills. Other areas of activity are the improvement of communication (via body language), the development of socio-emotional skills (showing emotional states), but also assistance with possible alterations in the home environment and the acquisition of suitable aids. The use of speech therapists as the third pillar of therapy serves primarily to train swallowing in order to enable independent food intake again. Frequent, targeted exercises are also aimed at restoring an improvement in the ability to speak in order to achieve more active communication with the patient’s environment.
Outlook and prognosis
The prognosis of locked-in syndrome is usually unfavorable. In most cases, symptoms persist throughout life or show only slight improvement over the lifespan. Achieving a complete recovery is rare. Nevertheless, the course of the disease depends on the cause of the disorders. If there is a possibility to eliminate the causal triggers, a cure can be achieved. Various therapies are used to support the quality of life and promote well-being.These are individually adapted to the possibilities of the organism and often vary over time. Locked-in syndrome results in long-term treatment of the patient. Without seeking medical care, at best the status quo is maintained. In an unfavorable case, premature demise of the affected person occurs. Many affected individuals report an improvement in their quality of life when they independently and on their own initiative perform targeted exercises and training outside of the therapy options offered. Nevertheless, most patients are dependent on the help of other people for the rest of their lives. It is usually not possible for them to manage their daily lives without full-time care. Due to the physical impairments, psychological sequelae may occur. The disease represents a strong emotional burden for the affected person but also for the relatives.
Prevention
To prevent a disease there are no special measures. A healthy lifestyle without body toxins such as alcohol, nicotine (and the accompanying substances contained in cigarettes) and drugs of any kind can minimize causes such as strokes and the like, but this is no guarantee.
Aftercare
Because self-healing usually cannot occur in locked-in syndrome, aftercare focuses primarily on managing severe limitations in movement. Most sufferers rely on the help and support of family and friends in their daily lives. The ability to speak may also be restricted, so that those affected can no longer speak properly or take food themselves. Since the disease often leads to psychological complaints, it can be helpful if those involved, including relatives, seek professional, psychological help. Exchanges with other affected persons in self-help groups can also bring about an exchange of valuable information and increase self-confidence in dealing with the disease.
What you can do yourself
The actions that sufferers of locked-in syndrome can take to improve their situation are limited because of the symptoms. Therefore, until appropriate therapy is initiated that allows at least partial movement and partial ambulation, affected individuals are completely dependent on their environment, with the exception of the ability to communicate. As therapy begins, it is also up to the affected person to consistently incorporate exercises that can be performed alone or in a private environment into his or her daily schedule. This is especially true when the inpatient stay ends, as this usually also means a reduction in therapy hours. For the person’s environment, the situation means that they must also learn certain forms of communication. Due to the restrictions, it becomes necessary to adapt communication in order to stay in contact with the affected person. At the same time, it is important not to speak in an oversimplified way – like a toddler, for example – because although Locked-In-Syndrome patients seem objectively helpless, their perception is usually not impaired. It is also incumbent on relatives to support the care of the affected person. This includes visits, specially performed hand movements (if permitted) and, of course, checking for possible bedsores or poor posture. Further measures that can be taken by the affected person and their environment are very dependent on a possible therapeutic success and the late effects of the locked-in syndrome. They belong accordingly to be worked out together with physicians and therapists.
