apallic syndrome

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Classification according to ICD-10
G93.80 apallic syndrome
ICD-10 online (WHO version 2019)

The apallic syndrome is a clinical picture in neurology , which is caused by severe damage to the brain . This leads to a functional failure of the entire cerebral function or larger parts, while functions of the diencephalon , brain stem and spinal cord are retained. As a result, those affected appear awake, but in all probability have no consciousness and only very limited possibilities of communication (e.g. through concepts such as basic stimulation ) with their environment. In Germany, it is assumed that at least 10,000 people are affected - with at least 1,000 new patients per year.

Extensive synonyms are persistent vegetative state ( lat. Coma vigil ) and Lucid stupor .

definition

The Multi-Society Task Force on PVS defined diagnostic criteria for vegetative state in 1994 :

Development of the term

The term apallic syndrome was first used in 1940 by the German psychiatrist Ernst Kretschmer - derived from the Latin word for coat, ie “pallium”. The mantle refers to the cerebral cortex . In connection with the prefix "a" (for example: "un-", see also Alpha privativum ) the term apallic syndrome describes a condition without a cerebral mantle.

In 1940 Ernst Kretschmer described a patient with apallic syndrome as follows: “The patient lies awake with his eyes open. The gaze is staring or gliding back and forth without a point of fixation. Even the attempt to draw attention does not succeed, or at most in a trace, reflexive escape and defensive movements may be missing ... “ Addressing , touching, holding up objects does not arouse any recognizable echo. Jennet and Plum introduced the term persistent vegetative state in 1972 . Through the Multi-Society Task Force on PVS 1994 the distinction "persistent vegetative state" between (persistent was vegetative state) for an at least partially back-formable state and "permanent vegetative state" (permanent vegetative state) established permanent damage.

The term “vegetative state” refers to the fact that the autonomous (vegetative) nervous system maintains the basic vital functions such as breathing, circulation, digestion etc.

Since 2009, doctors have been calling the colloquial vegetative state syndrome unresponsive wakefulness (SRW). The term replaces the terms “permanent vegetative state” and “apallic syndrome”. At the SRW patients have opened their eyes, but do not show any externally recognizable excitement of consciousness. Vital functions such as breathing and digestion function independently, sometimes a sleep-wake rhythm is pronounced, but there are no specific movements or even communication.

causes

An apallic syndrome is always the result of severe damage to the brain . This is most often caused by a traumatic brain injury or a lack of oxygen (hypoxia) as a result of cardiac arrest . Furthermore, strokes , meningitis / encephalitis , brain tumors or neurodegenerative diseases (e.g. Parkinson's syndrome ) can lead to an apallic syndrome. Also massive persistent hypoglycaemia , z. B. after a suicide attempt with insulin , can cause the syndrome.

Ultimately, there is predominant damage to the cerebrum , with the loss of the cerebral cortex and B. bilateral damage to the thalamus or the reticular formation can lead to an apallic syndrome. Usually, however, there are mixed forms with damage to several important brain regions.

Symptoms

The apallic syndrome is usually the result of an acute serious illness (exception: neurodegenerative diseases). The patients are therefore mostly initially treated in an intensive care unit . During this time they are often comatose and have to be artificially ventilated and fed . Strong muscle twitching ( myoclonia ) often occurs after a lack of oxygen .

Thereafter, the physical functions stabilize. During this transition period of a few weeks, there is often massively increased blood pressure , sweating , palpitations , etc. as signs of a disorder of the autonomic nervous system . The symptoms are usually treated with appropriate medication . In contrast, independence from artificial ventilation is usually viewed as a sign of stabilization of the brain stem functions . The patient can then leave the intensive care unit. The wakefulness also mostly establishes itself during this period.

Ultimately, there can either be a more or less good recovery of the brain functions or the picture of a permanent vegetative state can develop. Those affected are often awake during the day, open their eyes without looking at anything, sometimes have certain movement patterns (e.g. template-like movements of the face or mouth ). The following phenomena are considered typical:

diagnosis

The diagnosis of an apallic syndrome is primarily made clinically, i.e. through personal examination and observation of the person affected. The examiner must have sufficient experience in assessing severe neurological defect syndromes. The observation period extends from weeks to months.

Apparatus-based diagnostics are useful as a support. This includes magnetic resonance imaging (MRI), electroencephalography (EEG) and evoked potentials (somatic evoked (SEP), possibly also acoustic evoked (AEP) and event-related potentials). Some of these enable the prognosis to be assessed in the early phase ( see below ). None of these examinations alone is suitable to make the diagnosis.

First and foremost, it is important to distinguish it from externally similar clinical pictures such as coma , locked-in syndrome or other treatable neurological or psychiatric diseases. With the appropriate experience, it is only difficult to distinguish it from a so-called syndrome of minimal consciousness (SMB, "minimally conscious state"), as there is a smooth transition here. It is also severe brain damage, but with basal, non-reflex behavioral patterns (e.g. visual fixation, eye movements or following simple prompts) or other conscious reactions (e.g. recognizing loved ones). Methods of functional magnetic resonance tomography (fMRI) and quantitative EEG as well as event-related potential and imaging methods can contribute to differentiation or prognosis .

However, misdiagnosis is not uncommon: A British study (Andrews et al., 1996) with 40 patients found that 43% of them were misdiagnosed with Apallic syndrome. These included seven patients who had been misdiagnosed for over a year, three of whom had been diagnosed for over four years. When the diagnosis was corrected, all of them had sufficient cognitive function to communicate wishes about their living conditions to the nursing staff.

therapy

The treatment is based on the phases of neurological early rehabilitation . The focus is initially on acute treatment (phase A). During this time, an incision in the windpipe ( tracheotomy ), a feeding tube through the abdominal wall ( PEG ) and often a urine drainage through the abdominal wall ( SPDK ) are usually made in order to secure vital functions and enable optimal nursing care (including nutrition). During this time, however, rehabilitation-oriented offers should be made using physiotherapy . This prevents contractures or pneumonia and improves swallowing. After the end of mechanical ventilation, the function of swallowing is decisive for whether the tracheostomy tube can be removed.

After completing the acute treatment, the early rehabilitation phase B follows. The range of therapies is expanded to include occupational therapy and neuropsychology . Music therapy and animal-assisted therapy can also be used. The aim is to improve motor, mental and psychological functions. The treatment must take place in a team under medical supervision, this is also requested and checked by the cost bearers. The concept of basal stimulation , which is intended to convey a perception of the environment adapted to the damage pattern and support simple body functions (e.g. movements) in an integrated educational and nursing concept , has become widely accepted .

In this phase, which lasts between a month and a year, the prognosis of the person affected is decided. If there is a noticeable improvement in physical and psychological performance, further phases of rehabilitation can be added (phases C / D / E). However, if he remains unconscious, phase F (permanent "activating treatment care") must be switched to.

Occupational therapy

Occupational therapists make a relevant contribution to the treatment of those affected in a vegetative state and therefore play an important role in the rehabilitation process. The occupational therapy follows a client-centered approach. This means that needs, roles, interests and the cultural background of the patient are integrated into the therapy. Occupational therapists define “involvement in an activity” as a basic human need . This means that every person has the right to carry out activities that are of great importance to the respective person.

In this way, at the beginning of the therapy, the patient's interests are found out through the relatives. A goal is then worked out together. Care is taken to ensure that meaningful goals for the patient are formulated with the help of the relatives.

A main task of occupational therapy is the individual support of those affected to be able to cope with their everyday lives . For example, occupational therapy works according to the Affolter model. In this model, the perceived search for information is supported by guiding those affected with perception problems . Occupational therapists are also responsible for the splint fitting for contracture prophylaxis, for positioning in bed and wheelchair, for preventing secondary consequential damage, and for wheelchair fitting / wheelchair adaptation.

Through discussions with relatives of patients in a vegetative state, activities and aids for the respective patient can be individually adapted to the environment and the needs of the patient and the relatives . Relatives have the opportunity to actively participate in occupational therapy interventions and to find their way around their special situation as relatives of an affected person in a vegetative state. In this way, occupational therapy can work specifically with relatives in order to offer them support in the new situation with weekly plans, checklists, further training and courses.

Discontinuation of therapy

This article or paragraph presents the situation in Germany . Help us to describe the situation in other countries.

In principle, everyone has the right to refuse such therapy in whole or in part and to want to die in such a case. As a person affected, he cannot represent his will because he is not capable of any expression of will. But it could at a time in which he still make a will and was able to express a decision in a living will have stuck. Otherwise, legal support (formerly guardianship ) must be set up for the person concerned . The task of the supervisor is to determine the presumed will of the person concerned. B. in discussions with close relatives and friends or previously treating therapists, in order to then present it to the currently treating doctors. If the presumed will of the person in care agrees with the doctor’s judgment, the presumed will after treatment or discontinuation of treatment can be complied with. If they do not match, the supervisory court must be called upon to make a decision, Section 1904 IV BGB. However, the euthanasia guidelines applicable in Germany must be adhered to. Since active euthanasia is forbidden, discussions about changing the law regularly arise both in the medical associations and among politicians. However, it is currently not foreseeable whether an adjustment will be made.

The term “therapy discontinuation” should be avoided. For terminally ill people, curative treatment takes a back seat to palliative care. Therefore, it is better to speak of a change in the therapy goal.

forecast

Overall, the chance of recovery from the apallic syndrome is far below 50%. The statistics are problematic because the diagnoses at the beginning were often not sufficiently reliable. The prognosis is more favorable for:

  • young people
  • traumatic brain damage (as opposed to hypoxia or ischemia )
  • short duration of coma at the beginning (<24 hours)

In addition, the chance of a cure is greater if the apallic syndrome was triggered by an external injury instead of an illness.

In contrast, there are several findings that speak for a very likely lack of improvement:

Individual cases should be treated first ( see above ). An improvement is almost impossible with non-traumatic brain damage after three months, with traumatic brain damage after twelve months. Even if the condition improves, the majority of those affected remain dependent on outside help for a lifetime.

Social

Relatives

Up to 70% of coma patients are cared for in the family at home. This seems all the more desirable as the line to the minimally conscious state cannot be drawn with absolute certainty and emotional reactions are most likely to be expected. With the appropriate professional support (outpatient care services), this is often physically and mentally manageable for the families.

Compulsory schooling

School attendance remains compulsory for children with apallic syndrome in Germany . However, due to the severity of the brain damage, regular school lessons are excluded.

communication

Although the vegetative state is actually characterized by a lack of awareness and the ability to express oneself, the findings of researchers who report on communicative approaches to these patients have increased in recent years ( cf. e.g. Zieger 2001). Communication presupposes perception and orientation, which in turn are essential components of consciousness. In non-verbal communication with people in the long-term coma-awake phase (> 18 months), the existence of such characteristics of consciousness could be demonstrated (cf. Herkenrath 2006). Studies by a British research group have recently even provided evidence that some vegetative coma patients are aware of themselves and their surroundings (Owen et al. 2006). In recent years, Niels Birbaumer (2005) and his working group have been able to find indications that the quality of life of people in a vegetative state is probably much higher than one suspects “from the outside”.

Movies

  • SWR: The special learning - because the soul knows no coma.
  • Arte: The healing language of horses.

See also

literature

Broadcast reports

Web links

Wiktionary: Wachkoma  - explanations of meanings, word origins, synonyms, translations

Individual evidence

  1. ↑ Frank A. Miltner: Living wills in the vegetative state: Ways to more security. at: Informationsdienst Wissenschaft e. V.
  2. ↑ Raimund Firsching, Andreas Ferbert : Traumatic damage to the nervous system W. Kohlhammer Verlag, 2007, ISBN 978-3-17-019180-8 , p. 129.
  3. ↑ Klaus von Wild, Steven Laureys, Giuliano Dolce: Apallic syndrome, vegetative state: Inappropriate terms . In: Deutsches Ärzteblatt . tape 109 , no. 4 . Deutscher Ärzte-Verlag , January 27, 2012, p. A-143 / B-131 / C-131 ( aerzteblatt.de ).
  4. ↑ a b Andreas Bender, Ralf J. Jox, Eva Grill, Andreas Straube, Dorothée Lulé: vegetative state and minimal state of consciousness: systematic review and meta-analysis of diagnostic procedures . In: Deutsches Ärzteblatt International . No. 112 , 2015, p. 235–242 , doi : 10.3238 / arztebl.2015.0235 ( aerzteblatt.de ).
  5. ↑ a b c d R. Munday: Vegetative and minimally conscious states: How can occupational therapists help? Ed .: Neuropsychological rehabilitation. tape 15 , 2005, pp. 503-513 .
  6. ↑ a b c C. Cunningham, R. Wensley, D. Blacker, J. Bache, C. Stonier: Occupational therapy to facilitate physical activity and enhance quality of life for individuals with complex neurodisability . Ed .: British Journal of Occupational Therapy. tape 75 , no. 2 , 2012, p. 106-110 .
  7. ↑ M. Mastos, K. Miller, A. Eliasson, C. Imms: Goal-directed training: linking theories of treatment to clinical practice for improved functional activities in daily life . Ed .: Clinical Rehabilitation. tape 21 , 2007, p. 47-55 .
  8. ^ A. Häggström, M. Larsson Lund: The complexity of participation in daily life: A qualitative study of the experiences of persons with acquired brain injury . Ed .: Journal of Rehabilitation Medicine. tape 40 , 2008, p. 89-95 .
  9. ↑ The Affolter-Modell®: Models, Successes, History. Perception.ch Foundation, 2017, accessed on May 21, 2017 .
  10. ↑ The apallic syndrome from an ethical and legal point of view. Retrieved September 26, 2018 .