Being a prisoner of your own body – a terrible idea that becomes an oppressive truth in the case of locked -in syndrome. The best-known media example of today is probably Stephen Hawking.
What is locked-in syndrome?
According to Foodezine, the 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 people to communicate with their environment.
Those affected can usually only communicate via eye movements (winking, blinking, etc.), but even in this way only very limited statements are possible through yes/no questions (or and/or questions).
If this possibility of communication also fails, help can only be provided by technical means in order to continue to maintain active contact with the outside world.
However, it should be noted that this disease is by no means a comatose state, since the patient has all of his consciousness at his disposal, i.e. he can hear, see and understand his environment.
The most common cause of this debilitating disease is brainstem infarction. The blood supply to the midbrain, brain bridge and extended spinal cord is so severely restricted or sometimes completely interrupted that there are considerable restrictions in various bodily functions.
Other common causes are meningitis (inflammation of the meninges), special nerve diseases (e.g. amyotrophic lateral sclerosis ), strokes and severe trauma and accidents. The locked-in syndrome can rarely be observed in patients with multiple sclerosis, arterial/ nerve inflammation or after abuse of toxic substances/drugs (heroin).
Symptoms, Ailments & Signs
The locked-in syndrome is associated with an intact state of consciousness with an almost complete inability to act. Those affected perceive stimuli. So you can hear, smell, taste, see and also (to a limited extent) feel. Language comprehension is usually not restricted.
The paralysis that occurs in locked-in syndrome involves the four extremities and horizontal gaze movements. In most cases, the ability to speak, swallow and facial expressions is lost. 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 with the situation of completely paraplegic people.
Those affected are not restricted in their alertness. In the broadest sense, they live through an ordinary biorhythm. There is hardly any pain or physical discomfort. The awareness of one’s own paralysis is there. The cognitive possibilities are usually only limited 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 differentiated from the vegetative state. In this case, it is questionable whether and to what extent those affected perceive their surroundings.
Diagnosis & History
The diagnosis of LiS cannot be made simply by “taking a look” because the clinical picture is very similar to a vegetative state or akinetic mutism (a disease that is characterized above all by a severe drive disorder).
Appropriate diagnostic methods are primarily electrical and magnetic measurements of brain and muscle activity. CT and MRT can thus be used to determine changes in blood flow and metabolism in the brain. These technical diagnostic methods are usually combined with laboratory techniques, for example to be able to better assess an inflammatory state in meningitis.
The course of this disease is very individual and depends on both medical care and the cause of the outbreak. It can be assumed that there is a mortality rate of 59-70% if the LiS was triggered by bleeding or blockage in cerebral vessels. For trauma, tumors, etc. this rate drops to around 30%. Diseases caused by toxins (poisons/drugs) almost never lead to death.
As a rule, those affected suffer from significant psychological complaints and complications as a result of the locked-in syndrome. However, they cannot express themselves to the outside world and cannot communicate with them. This leads to clear and considerable restrictions in the everyday life of the person concerned. The patients themselves usually suffer from paralysis with locked-in syndrome and are therefore dependent on the help of other people in their everyday life.
This often leads to restricted mobility, so that the patients are dependent on a wheelchair. Due to the speech disorders, communication with the outside world is usually lost. Those affected are in a coma and suffer from severe depression and other mental disorders.
In most cases, the life expectancy of the patient is not reduced by the locked-in syndrome. However, the further course depends heavily on the cause of the locked-in syndrome, so that a general course of the disease cannot be predicted. A causal treatment of the locked-in syndrome is usually not possible.
Those affected are dependent on various therapies and help in everyday life. As a rule, the syndrome cannot be completely cured either. Above all, the patient’s relatives suffer from significant depression and other psychological limitations as a result of the syndrome.
When should you go to the doctor?
By definition, locked-in syndrome prevents the sufferer from going to the doctor himself. However, the worrying symptoms always lead to the patient being hospitalized. Since stroke is the most common trigger of locked-in syndrome, medical supervision usually arises after the incident.
Those affected by locked-in syndrome generally do not have the opportunity to do without medical attention. This is because there is an urgent need to differentiate the condition from other states of incapacity and provide appropriate care and support. Since the person concerned cannot communicate effectively and the symptoms of the condition can be confused so easily, it is sometimes up to the relatives to point out the possibility of a locked-in syndrome.
Since the disease requires a great deal of medical attention, neurologists who check the functionality of the body are particularly important as it progresses. For the course of a possible recovery, it is important that the physiotherapeutic, logopaedic, ergotherapeutic and, if necessary, psychotherapeutic treatment is optimally covered by specialists.
Treatment & Therapy
Treatment of those affected primarily requires one thing: an intensive and individual combination of ergotherapy, speech therapy and physiotherapy. The main goal is to mobilize the patient and thus free him from his immobility. The sooner such rehabilitation is scheduled, the more likely it is to be successful.
In physiotherapy, the principle of “systematic repetitive basic training” is primarily used today. This means that initially only individual, small movements of the joints are trained. If these can be carried out independently again and certain positions can be held, the training exercises are extended to several joints and muscle groups and later practiced in precise activities (e.g. holding a fork and bringing it to the mouth).
Occupational therapy offers further assistance in relearning various skills, with the aim of rebuilding fine and gross motor skills. Other areas of responsibility are the improvement of communication (via body language), the development of socio-emotional skills (showing emotional states) but also assistance with any changes in the home environment and the purchase of suitable aids.
The use of speech therapists as the third pillar of therapy is primarily used for swallowing training in order to enable independent food intake again. Frequent, targeted exercises are also intended to improve language skills in order to achieve more active communication with the patient’s environment.
Outlook & Forecast
The prognosis of locked-in syndrome is usually unfavorable. In most cases, the symptoms last for life or show only slight improvements over the lifespan. Achieving a full recovery is rare. Nevertheless, the course of the disease depends on the cause of the disorders. If there is a way to fix the causative triggers, a cure can occur.
Various therapies are used to support 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 involves long-term treatment of the patient. Without the use of medical care, the status quo is maintained at best. In the worst case, the victim dies prematurely.
Many of those affected report an improvement in their quality of life if they carry out targeted exercises and training independently and on their own initiative outside of the therapy options offered. Nevertheless, most patients depend on the help of other people throughout their lives. It is usually not possible for them to cope with their everyday life without full-time care. The physical impairments can lead to psychological complications. The disease is a heavy emotional burden for the person affected as well as for their relatives.
There are no special measures to avoid illness. A healthy lifestyle without body toxins such as alcohol, nicotine (and the accompanying substances contained in cigarettes) and drugs of any kind can cause strokes and the like. minimised, but this is no guarantee.
Because locked-in syndrome is not usually self-healing, follow-up care focuses primarily on managing the severe limitations in movement. Most of those affected depend on the help and support of family and friends in their everyday life. The ability to speak can also be restricted, so that those affected can no longer speak properly and can no longer eat themselves.
Since the disease often leads to psychological problems, it can be helpful if those involved, including relatives, seek professional psychological help. The exchange with other affected people in self-help groups can also bring about an exchange of valuable information and self-confidence in dealing with the disease.
You can do that yourself
The actions that people with locked-in syndrome can take to improve their situation are limited because of the symptoms. Until a suitable therapy is applied, which enables at least partial movements and partial movement sequences, those affected are completely dependent on their environment – with the exception of the possibility of communication.
When therapy begins, it is also up to those affected to consistently incorporate exercises that can be carried out alone or in their private environment into their daily schedule. This applies in particular when the inpatient stay is ended, as this usually also means a reduction in therapy hours.
For the environment of the person concerned, the situation means that they also have to learn certain forms of communication. Due to the restrictions, it becomes necessary to adapt communication in order to stay in touch with the person concerned. At the same time, the language should not be overly simplified – appropriate for a small child, for example – since locked-in syndrome patients appear objectively helpless, but their perception is usually not restricted. It is also the responsibility of relatives to support the care of the person concerned. This includes visits, specially performed maneuvers (where permitted) and of course checking for possible bed sores or bad posture.
Further measures that can be taken by the person concerned and their environment are very dependent on the possible success of the therapy and the late effects of the locked-in syndrome. They should be worked out together with doctors and therapists.