Aphnetic Mutism: Symptoms, Causes and Treatment

He Akinetic mutism Or greater abulia is a subjective lack of thought, in which the person is not able to initiate alone any movement or even speech. For example, this patient, although thirsty, may be sitting in front of a glass of water without drinking from it.

This may be due to damage in Brain structures That seem to handle the motivation to conduct behaviors, being immersed in an important state of apathy .

We can define akinetic mutism as a decrease or absence of spontaneous behaviors despite the fact that motor skills are intact since the origin of the problem is, as we said, a motivational one (affects the dopaminergic circuits of the brain).

It is a syndrome difficult to diagnose since it can be part of altered states of consciousness. And sometimes it appears as a continuum with the akinetic mutism located between the coma and the return to wakefulness.

The case of Emilio

Rodríguez, Triviño, Ruiz and Arnedo (2012) described a curious case of a patient who, after several brain surgical interventions, presented what is defined as"the mind in white".

The patient, who will be called"Emilio", was 70 years old when a benign tumor (meningioma) was detected in the cerebral cortex. The patient felt that he had difficulty naming objects and describing situations, as well as motor clumsiness when playing the saxophone, a task he had previously performed without difficulty since he played in the band of his town.

He also liked to take care of his garden and was beginning to have problems that he did not have before.

He was Craniotomy To eliminate the tumor that passed without complications. One year later, in a review, several tumor nodules were detected, so that this patient had to undergo multiple surgeries and Radiosurgery Over 6 years.

This gave rise to different complications, since Emilio got to present right hemiparesis (it is a frequent condition after a brain injury in which the right side of the body weakens) and motor difficulties of which he recovered with treatment.

However, another magnetic resonance Manifested a new tumor that occupied the anterior cingulate cortex. After surgery again to remove it, the patient was evaluated, diagnosing his condition as akinetic mutism.

Causes of akinetic mutism

The most common cause of akinetic mutism is vascular, although there are some cases whose origin is the exposure or intake of toxins, infections or degenerative processes.

Vascular lesions that cause this disease cause heart attacks in:

- The anterior cerebral artery, which impairs the anterior cingulate cortex and parts of the frontal lobe.

In addition, it not only appears by lesions in the anterior cingulate cortex, but also by the damage in the connections of frontal areas with subcortical zones.

To understand the origin of this disorder, it is important to note that one of the major areas that receives Dopamine Of the meso-cortical dopaminergic system, as it receives information from deeper areas of the brain that make up the famous brain reward system.

This system is essential for conducting survival-motivating behaviors such as perpetuating the species or finding food. Therefore, it is not surprising that if dopaminergic circuits are damaged, a State of apathy .

- The paramedian thalamic arteries.

- Arteries that supply the basal ganglia: damage to the frontal-basal connections of the brain will isolate the frontal areas of structures such as the caudate nucleus, pale balloon, putamen or internal capsule, which are very important for the person to find motivation for Conduct behaviors.

- or infarct in arteries of the cerebellum Which damage the back of the cerebellum and the vermis area. It has been found that the cerebellum may be associated with functions such as verbal fluency, working memory, emotions or task planning (interestingly, very typical of the frontal lobe). Anyway, more research is needed to know exactly how it manifests in akinetic mutism.

In conclusion, structures damaged in akinetic mutism seem to be involved in the initiation and maintenance of behavior, in addition to the motivation to trigger it. What do we mean by motivation?

In this context, it is defined as the energy needed to achieve something that is desired or avoided something aversive and that is influenced by the emotional state (Stuss and Benson, 1986). It is as if the will was lacking and the person could not be put in place to cover their needs, remaining all the time quiet and silent.

This is why it is called"having the mind blank"(Rodríguez et al., 2012). In fact, Damasio (1999) describes that patients who have recovered from akinetic mutism, when asked why they did not speak when they had the disease said" Is that nothing came to mind ".

symptom

The most common and distinctive symptoms are:

- Failure to initiate spontaneous voluntary actions.

- They remain quiet, inactive throughout the day (akinesia). They only perform automatic behaviors.

- Silence and lack of gesticulation (for example, they do not indicate signs that they are listening or understanding what others are saying)

- If there is speech, it is very scarce and it is characterized by hypophony (low volume of voice), and by drag of words. Pronunciation and syntax are often correct, as long as there is no damage to brain structures dedicated to language.

- Yes they understand what is asked, but it does not seem at first sight, since when they respond they do it coherently. They respond mainly when asked biographical data, such as their name or date of birth. If they are other types of questions, they prefer to answer with"yes","no"or monosyllables.

- They do not usually respond if the questions are open or imply emotional or affective content.

- Normally they do not initiate conversations, they do not ask questions, they do not even make requests with respect to their basic necessities: to eat, to drink, to go to the bathroom. They do not express what they want or seem to do anything to achieve it.

- It often happens that they can only take action if another person helps them initiate them. They can use the objects without any problem, but never initiate the movement of their own volition. According to the example that we put before the glass of water, Emilio if he was thirsty he did not drink until another person put the glass in the hand.

- Motor Perseverations: means performing repetitive motor actions without a goal. For example, in Emilio's case, he continually folded the end of his shirt with his fingers. Which indicates that there are no problems in the realization of movements, but in the will to start them.

- Another distinctive symptom is that these patients before a stimulus that is harmful can"wake up", that is, react by shaking and even emitting words (Godefroy, 2013).

- As for emotional states, they appear to be variable in each case. Some exhibit almost imperceptible emotional expressions while others have significant alterations, sometimes typical of frontal brain damage such as impulsive and uninhibited emotional outbursts.

However, the symptoms may vary depending on the functional deficits that cause each affected brain area.

Types

Two types of akinetic mutism have been defined depending on where the lesions in the brain are and the symptoms it causes:

Front akinetic mutism

It is the most common and is associated with unilateral or bilateral focal lesions of the anterior cingulate cortex.

If this lesion is unilateral, the patients usually recover a few weeks later, but if bilateral it will present a total loss of the onset of spontaneous behavior that is not reversible. Sometimes, damage can also be extended to the supplemental motor area causing movement deficits.

Diencephalic-mesencephalic akinetic mutism

It is due to the affectation of the Diencephalon , Especially the upward activating reticular system. This type presents a lower vigilance than the mutism of the frontal type and also distinguishes itself from this one in that the patient presents paralysis of the vertical gaze.

Differential diagnosis

As we said, it is difficult to detect because it is difficult to evaluate because patients have a difficult time responding to the tests and have to be ingenuárselas for an effective neuropsychological evaluation. For this reason it is easy to confuse akinetic mutism with other states or disorders.

Therefore, care must be taken not to confuse with:

• Vegetative state: Unlike akinetic mutism, in the vegetative state there is what is called coma vigil, a state in which the patient can not follow external visual stimuli with the eyes, even if they are open; They can not express themselves or follow simple orders. They do retain some reflexes, but they can not perform behaviors because they would need to process with more cortical brain structures that patients with akinetic mutism have intact.
• State of minimum conscience In akinetic mutism, is not answered due to a severe state of abulia and apathy that causes it does not move or speak spontaneously; But unlike the least consciousness, if they can emit coherent answers when they are incited and initiate movements when they are helped.
• Captivity Syndrome The movement is not caused by limb paralysis caused by damage to the spinal and corticobulbar tracts, leaving most of the cognitive functions, vertical eye movements and blinking (frequently used to communicate) intact.
• Aphasia It can be difficult to make a distinction, since in some cases you may have akinetic mutism and aphasia at the same time. The main difference is that the initiative and motivation to communicate is preserved in aphasics, whereas patients with akinetic mutism lack these.
• Abulia: Would be at a level immediately below the akinetic mutism, being lighter.
• Depression .

Rehabilitation

What should be the goals of rehabilitation?

- The main, reduce apathy. Apathy is characterized by an alteration in the ability to establish goals, lack of motivation, loss of initiative and spontaneity, affective indifference. It is also commonly associated with a lack of awareness of the disease, which has a very negative impact on the person's life and overall neuropsychological functioning. It is necessary to reduce that apathy and increase patient collaboration for satisfactory rehabilitation.

- Maximize your independence.

- As in the case of Emilio, the family usually asks to be able to perform the Activities of the daily life that before did with normality.

Aspects to take into account for rehabilitation (Sanz and Olivares, 2013)

The Neuropsychological Rehabilitation Is the application of intervention strategies that seek to enable patients and families to reduce, cope with or manage cognitive deficit.

To do this, we will work directly improving the performance of cognitive functions through repetition of exercises.

Deficits can be intervened in 3 ways:

• By restoration (direct training, restore damaged function).
• Through compensation (using the skills that are intact to minimize the negative consequences of those affected).
• By substitution (it is used when the two techniques mentioned are not possible, and it is a question of confronting the damages by teaching the affected to handle external signals and devices to minimize these limitations).

Important things to keep in mind:

• It is important to start rehabilitation as soon as possible.
• It is essential to develop an interdisciplinary work, with several professionals from different fields.
• In order for a neuropsychological intervention program to be effective, it must have a hierarchical organization of tasks according to its level of difficulty, arriving each time at a balance between the patient's abilities and the difficulty of the task.
• The main objectives will be self-care, independence and integration.
• Do not forget the emotional aspects.
• Adapt rehabilitation to be as generalizable as possible to everyday situations.
• Restructure the patient's environment if necessary (called environmental strategies).
• When you are in a more advanced phase of treatment, develop metacognitive strategies. That is, trying to have the patient acquire internal strategies that allow him to control his own attention, avoid being distracted by any stimulus, plan a sequence of tasks, use mnemonic rules, take decisions Properly, etc.

Treatment

• Pharmacological treatment: to reduce apathy, mainly dopaminergic agonists such as Levadopa Or bromocriptine, since the dopaminergic pathways are often affected.
• Getting a minimum level of patient collaboration is absolutely necessary to start working. You can start by raising awareness of the deficit, which means that we have to make the person realize that he has a problem and that he must make an effort to recover.
• Carry out family activities that are valuable to the person, that can"awaken"previously learned behaviors.
• It is essential for the family to collaborate in therapy, since they are the ones who spend most of the time with the patient. It is necessary to educate them to adequately manage the environment in which the patient lives, to structure the activities of daily life to make them simpler. It is appropriate that they help the patient initiate actions, trying to be motivating tasks, and that adapt to the cognitive level of the affected.
• It is useful to ask family, friends, what the patient liked to do before, what motivated him, what hobbies he had, etc. That way we can better know the affected and develop therapeutic activities that motivate and be pleasant.
• Break down the activities in small steps and with clear instructions on their implementation. When you do it correctly, you will always be Feedback Immediately after each step. It is appropriate to ensure that failure is not produced so that it does not become frustrated.
• Start training activities related to basic needs such as eating, drinking, going to the service... to increase the autonomy of the patient as soon as possible.
• The patient is more likely to respond or issue any behavior if given a choice between two alternatives.
• It is best to give clear and firm orders.
• Do not saturate the person with activities, as they can get tired and thus there is a very common confusion between apathy and fatigue.
• The emotional support of the family is very important: they must make the patient feel that they are willing to help him, expressing affection (but never treat the patient with grief or as if he were a child) and not lose hope. Try to visualize the situation as hopeful, giving the affected person an understanding that the situation will undoubtedly improve. Give positive expectations of the future, avoid showing crying and complaining in front of the patient because it could sink you. (Carrión, 2006).
• Show the family and the patient the progress, no matter how slight.
• The patient should feel that his life is gradually becoming normalized: it is okay to have a routine, but it is not essential to lock yourself in the house. It is a positive thing to visit friends and try to take you to environments where you previously attended.
• The"Phone Effect": Yarns & Quinn (2013) describe a startling case of a patient with akinetic mutism who began talking through a phone call with his wife. This patient spoke and answered questions satisfactorily over the phone, but in person presented more difficulties. After a while it was observed that little by little verbal interaction was improving in all areas, becoming generalized. It seems to be effective provided it is accompanied by pharmacological treatment.
• Behavioral strategies: backward chaining: decompose the task into steps and ask the patient to take the last step. To do this, first complete task is done (for example, brushing teeth), grasping the patient's arm and doing all the movements. Then the task is repeated with help, but the last step should be done by the patient alone (dry mouth). Encourage him to do it"now you must dry your mouth with the towel, come"and strengthen him when he does. Then the task is repeated until the patient can brush his teeth without any help. It has been shown that this technique is very useful for patients with motivation problems.
• Task analysis: It consists of dividing a task into small and sequential steps and writing them into a list. This allows verifying that each case is completed. This technique makes it much easier to start, finish and monitor the activity. In addition, it reduces fatigue, so it consumes less energy because the patient should not plan, organize and remember the steps necessary to reach a goal. It is very useful to establish a routine of the activities that must be done daily, since if they are repeated consistently they can become automatic habits.
• In a second moment, another strategy is developed to increase the frequency of desirable but infrequent behaviors, rewarding their accomplishment with very pleasant consequences for the patient. To do this, a list must be made of what the patient is known to like and another list of what is expected to be done to achieve it. To find out if it is useful for the patient (because the family normally completes it), he should evaluate each point in the list from 1 to 10 depending on the degree of difficulty or, depending on the degree of enjoyment that he produces.

References

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