Akinetic mutism is a medical term describing patients tending neither to move (akinesia) nor speak (mutism). Akinetic mutism was first described in 1941 by Cairns et al. as a mental state where patients lack the ability to move or speak.[1] However, their eyes may follow their observer or be diverted by sound.[1] Patients lack most motor functions such as speech, facial expressions, and gestures, but demonstrate apparent alertness.[2] They exhibit reduced activity and slowness, and can speak in whispered monosyllables.[1][3] Patients often show visual fixation on their examiner, move their eyes in response to an auditory stimulus, or move after often repeated commands.[1][2] Patients with akinetic mutism are not paralyzed, but lack the will to move.[1] Many patients describe that as soon as they ‘will’ or attempt a movement, a ‘counter-will’ or ‘resistance’ rises up to meet them.[4]

Akinetic mutism varies across all patients. Its form, intensity, and clinical features correspond more closely to its functional anatomy rather than to its pathology. However, akinetic mutism most often appears in two different forms: frontal and mesencephalic.[2]

Frontal akinetic mutismAkinetic mutism can occur in the frontal region of the brain and occurs because of bilateral

frontal lobe damage. Akinetic mutism as a result of frontal lobe damage is clinically characterized as hyperpathic.[5] It occurs in patients with bilateral circulatory disturbances in the supply area of the anterior cerebral artery.[2]

Mesencephalic akinetic mutismAkinetic mutism can also occur as a result of damage to the mesencephalic region of the brain.

Mesencephalic akinetic mutism is clinically categorized as somnolent or apathetic akinetic mutism.[5] It is characterized by vertical gaze palsy and ophthalmoplegia. This state of akinetic mutism varies in intensity, but it is distinguished by drowsiness, lack of motivation, hyper-somnolence, and reduction in spontaneous verbal and motor actions.[2][5]

SymptomsSymptoms of akinetic mutism progress over time.[2] The occurrence of akinetic mutism takes

place approximately four months after the symptoms first appear.[2]

Lack of motor function (but not paralysis)[1]

Lack of speech [1]

Apathy[6]

Slowness[6]

Disinhibition [3] Causes

Many cases of akinetic mutism have occurred after a thalamic stroke.Akinetic mutism can be caused by a variety of things. It often occurs after brain injury or as a symptom of other diseases.

Frontal lobe damageAkinetic mutism is often the result of severe frontal lobe injury in which the pattern of

inhibitory control is one of increasing passivity and gradually decreasing speech and motion.Thalamic strokeMany cases of akinetic mutism occur after a thalamic stroke.[3] The thalamus helps regulate

consciousness and alertness.Ablation of cingulate gyrusAnother cause of both akinesia and mutism is ablation of the cingulate gyrus. Destruction of

the cingulate gyrus has been used in the treatment of psychosis. Such lesions result in akinesia, mutism, apathy, and indifference to painful stimuli.[7] The anterior cingulate cortex is thought to supply a "global energizing factor" that stimulates decision making.[8] When the anterior cingulate cortex is damaged, it can result in akinetic mutism.

Other

Akinetic mutism is a symptom during the final stages of Creutzfeldt-Jakob Disease (a rare degenerative brain disease) and can help diagnose patients with this disease.[2][9] It can also occur in a stroke that affects both anterior cerebral artery territories. Another cause is neurotoxicity due to exposure to certain drugs such as tacrolimus and cyclosporine.

Other causes of akinetic mutism are as follows: Respiratory arrest and cerebral hypoxia [6]

Acute cases of encephalitis lethargica [3] Meningitis [3] Hydrocephalus [3] Trauma[3]

Tumors[3]

Aneurysms [3]

Olfactory groove meningioma Cyst in third ventricle [1]

Toxical lesions and infections of central nervous system [10]

Delayed post-hypoxic leukoencephalopathy (DPHL) [6]

Creutzfeldt-Jakob Disease (mesencephalic form) [2]

Diagnosis and treatmentAkinetic mutism can be misdiagnosed as depression, delirium, or locked-in syndrome, all of

which are common following a stroke.[3] Patients with depression can experience apathy, slurring of speech, and body movements similar to akinetic mutism. Similarly to akinetic mutism, patients with locked-in syndrome experience paralysis and can only communicate with their eyes.[3] Correct diagnosis is important to ensure proper treatment. A variety of treatments for akinetic mutism have been documented, but treatments vary between patients and cases.

Magnesium sulfateTreatments using intravenous magnesium sulfate have shown to reduce the symptoms of

akinetic mutism. In one case, a 59-year-old woman was administered intravenous magnesium sulfate in an attempt to resolve her akinetic mutism. The patient was given 500 mg of magnesium every eight hours, and improvement was seen after 24 hours. She became more verbal and attentive, and treatment was increased to 1000 mg every eight hours as conditions continued to improve.[11]

Cyst punctureAs seen in the case of Elsie Nicks, the puncture or removal of a cyst causing akinetic mutism

can relieve symptoms almost immediately. However, if the cyst fills up again, the symptoms can reappear.[1]

Dopamine agonist therapySymptoms of akinetic mutism suggest a possible presynaptic deficit in the nigrostriatal

pathway, which transmits dopamine. Some patients with akinetic mutism have shown to improve with levodopa or dopamine agonist therapy,[12] or by repleting dopamine in the motivational circuit with stimulants, antidepressants, or agonists such as bromocriptine or amantadine.[6]

Other treatments include amantadine, carbidopa-levodopa, donepezil, memantine, and oral magnesium oxide.[6][11]

History Fourteen-year-old Elsie Nicks was the first patient to be diagnosed with akinetic mutism by

Cairns in 1941. She suffered from severe headaches her entire life and was eventually given morphia to help with treatment. She began to enter a state of akinetic mutism, experiencing apathy and loss of speech and motor control. A cyst on her right lateral ventricle was tapped, and as soon as the needle advanced toward the cyst, she let out a loud noise and was able to state her name, age, and address. After her cyst was emptied, she regained her alertness and intelligence, and she had no recollection of

her time spent in the hospital. The cyst was drained two more times over the next seven months and was eventually removed. After eight months of rehabilitation, Elsie no longer experienced headaches or akinetic mutism symptoms.[1]

See also Selective mutism Locked-in syndrome Athymhormic syndrome Aboulia

Selective mutismSelective mutism (SM) is an anxiety disorder in which a person who is normally capable of

speech does not speak in specific situations or to specific people. Selective mutism usually co-exists with shyness or social anxiety.[1] People with selective mutism stay silent even when the consequences of their silence include shame, social ostracism or even punishment.[2]

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Children and adults with selective mutism are fully capable of speech and understanding language but fail to speak in certain situations, though speech is expected of them.[3] The behaviour may be perceived as shyness or rudeness by others. A child with selective mutism may be completely silent at school for years but speak quite freely or even excessively at home. There is a hierarchical variation among people with this disorder: some people participate fully in activities and appear social but do not speak, others will speak only to peers but not to adults, others will speak to adults when asked questions requiring short answers but never to peers, and still others speak to no one and participate in few, if any, activities presented to them. In a severe form known as "progressive mutism", the disorder progresses until the person with this condition no longer speaks to anyone in any situation, even close family members.

Selective mutism is by definition characterized by the following:[4]

Consistent failure to speak in specific social situations (in which there is an expectation for speaking, e.g., at school) despite speaking in other situations.

The disturbance interferes with educational or occupational achievement or with social communication.

The duration of the disturbance is at least 1 month (not limited to the first month of school). The failure to speak is not due to a lack of knowledge of, or comfort with, the spoken

language required in the social situation. The disturbance is not better accounted for by a communication disorder (e.g., childhood-

onset fluency disorder) and does not occur exclusively during the course of autism spectrum disorder, schizophrenia, or another psychotic disorder.Selective mutism is strongly associated with anxiety disorders, particularly social anxiety

disorder. In fact, the majority of children diagnosed with selective mutism also have social anxiety disorder (100% of participants in two studies and 97% in another).[5][6][7] Some researchers therefore speculate that selective mutism may be an avoidance strategy used by a subgroup of children with social anxiety disorder to reduce their distress in social situations.[8][9]

Particularly in young children, SM can sometimes be confused with an autism spectrum disorder, especially if the child acts particularly withdrawn around his or her diagnostician, which can lead to incorrect treatment. Although autistic people may also be selectively mute, they display other

behaviors—hand flapping, repetitive behaviors, social isolation even among family members (not always answering to name, for example)—that set them apart from a child with selective mutism. Some autistic people may be selectively mute due to anxiety in social situations that they do not fully understand. If mutism is entirely due to autism spectrum disorder, it cannot be diagnosed as selective mutism as stated in the last item on the list above.

Selective mutism may co-exist with or cause the child to appear to have attention deficit disorder. Many people with the inattentive form of ADHD show little or no interest in other people. People with inattentive ADHD may appear to be "space cadets" or "out in their own world", and may be slower to respond to social stimuli. Children with selective mutism, especially when they have severe social anxiety, may also display this behavior. In addition, many children with selective mutism are highly sensitive, and they may be distracted from the task at hand by sensory input or their anxiety.[citation needed]

The former name elective mutism indicates a widespread misconception among psychologists that selective mute people choose to be silent in certain situations, while the truth is that they often wish to speak but cannot. To reflect the involuntary nature of this disorder, the name was changed to selective mutism in 1994.

The incidence of selective mutism is not certain. Due to the poor understanding of this condition by the general public, many cases are likely undiagnosed. Based on the number of reported cases, the figure is commonly estimated to be 1 in 1000, 0.1%.[10] However, a 2002 study in The Journal of the American Academy of Child and Adolescent Psychiatry estimated the incidence to be 0.71%.[11]

Other symptoms[edit]Besides lack of speech, other common behaviors and characteristics displayed by selectively

mute people include:[12][13]

Shyness, social anxiety, fear of social embarrassment, and/or social isolation and withdrawal Difficulty maintaining eye contact Blank expression and reluctance to smile Stiff and awkward movements Difficulty expressing feelings, even to family members Tendency to worry more than most people of the same age Desire for routine and dislike of changes Sensitivity to noise and crowds

On the positive side, many people with this condition have: Above-average intelligence, perception, or inquisitiveness Creativity and a love for art or music Empathy and sensitivity to others' thoughts and feelings A strong sense of right and wrong[13]

CausesSelective mutism (SM) is an umbrella term for the condition of otherwise well-developed

children who cannot speak or communicate under certain settings. The exact causes that affect each child may be different and yet unknown. There have been attempts to categorize, but there are no definitive answers yet due to the under-diagnosis and small/biased sample sizes. Many people are not diagnosed until late in childhood only because they do not speak at school and therefore fail to accomplish assignments requiring public speaking. Their involuntary silence makes the condition harder to understand or test. Parents often are unaware of the condition since the children may be functioning well at home. Teachers and pediatricians also sometimes mistake it for severe shyness or common stage fright.

Selective mutism occurs in all racial and ethnic groups. The majority of reported cases are of white and interracial children. However this could be due to under-diagnosis and under-reporting in other ethnic groups.

Most children with selective mutism are hypothesized to have an inherited predisposition to anxiety. They often have inhibited temperaments, which is hypothesized to be the result of over-excitability of the area of the brain called the amygdala.[14] This area receives indications of possible threats and sets off the fight-or-flight response. Given the very high overlap between social anxiety disorder and selective mutism (as high as 100% in some studies[5][6][7]), it is possible that social anxiety disorder causes selective mutism.

Some children with selective mutism may have trouble processing sensory information. This would cause anxiety and a sense of being overwhelmed in unfamiliar situations, which may cause the child to "shut down" and not be able to speak (something that some autistic people also experience). Many children with selective mutism have some auditory processing difficulties.

About 20–30% of children with SM have speech or language disorders that add stress to situations in which the child is expected to speak.[15]

Despite the change of name from "elective" to "selective", a common misconception remains that a selectively mute child is defiant or stubborn. In fact, children with SM have a lower rate of oppositional behavior than their peers in a school setting.[16] Some previous studies on the subject of selective mutism have been dismissed as containing serious flaws in their design. According to a more recent systematic study it is believed that children who have selective mutism are not more likely than other children to have a history of early trauma or stressful life events.[17] Another recent study by Dummit et al., in 1997 did not find any evidence of trauma in their sample of children. Recent evidence has shown that trauma doesn't explain why most children with selective mutism develop the condition.[18] Many children who have Selective Mutism almost always speak confidently in some situations. Children who have experienced trauma however are known to suddenly stop speaking.

TreatmentContrary to popular belief, people with selective mutism do not necessarily improve with age.

[19] Effective treatment is necessary for a child to develop properly. Without treatment, selective mutism can contribute to chronic depression, further anxiety, and other social and emotional problems.[20][21]

Consequently, treatment at an early age is important. If not addressed, selective mutism tends to be self-reinforcing. Others may eventually expect an afflicted child to not speak and therefore stop attempting to initiate verbal contact. Alternatively, they may pressure the child to talk, increasing their anxiety levels in situations where speech is expected. Due to these problems, a change of environment may be a viable consideration. However, changing school is worth considering only if the alternative environment is highly supportive, otherwise a whole new environment could also be a social shock for the individual and/or deprive them of any friends or support they have currently. Regardless of the cause, increasing awareness and ensuring an accommodating, supportive environment are the first steps towards effective treatment. Most often afflicted children don't have to change schools or classes and have no difficulty keeping up except on the communication and social front. Treatment in teenage or adult years can be more difficult because the afflicted individual has become accustomed to being mute.

The exact treatment depends on the person's age, any comorbid mental illnesses, and a number of other factors. For instance, stimulus fading is typically used with younger children because older children and teenagers recognize the situation as an attempt to make them speak, and older people with this condition and people with depression are more likely to need medication.[22]

Like other disabilities, adequate accommodations are needed for an afflicted child to succeed both at school and in the home. Under the U.S. federal law, the Individuals with Disabilities Education

Act (IDEA), children with the disorder qualify for services based upon the fact that they have an impairment that hinders their ability to speak, thus disrupting their education. This assistance is typically documented in the form of an Individual Educational Plan (IEP). Post-secondary accommodations are also available for people with disabilities.

Under another law, Section 504 of the Rehabilitation Act of 1973, public school districts are required to provide a free, appropriate public education to every "qualified handicapped person" residing within their jurisdiction. If the child is found to have impairments that substantially limit a major life activity (in this case, learning), the education agency has to decide what related aids or services are required to provide equal access to the learning environment.

Self-modelingAn afflicted child is brought into the classroom or the environment where the child will not

speak and is videotaped. First, the teacher or another adult prompts the child with questions that likely will not be answered. A parent, or someone the child feels comfortable speaking to, then replaces the prompter and asks the child the same questions, this time eliciting a verbal response. The two videos of the conversations are then edited together to show the child directly answering the questions posed by the teacher or other adult. This video is then shown to the child over a series of several weeks, and every time the child sees himself or herself verbally answering the teacher/other adult, the tape is stopped and the child is given positive reinforcement.

Such videos can also be shown to afflicted children’s classmates to set an expectation in their peers that they can speak. The classmates thereby learn the sound of the child’s voice and, albeit through editing, have the opportunity to see the child conversing with the teacher.[23][24]

Mystery motivatorsMystery motivation is often paired with self-modeling. An envelope is placed in the child’s

classroom in a visible place. On the envelope, the child’s name is written along with a question mark. Inside is an item that the child’s parent has determined to be desirable to the child. The child is told that when he or she asks for the envelope loudly enough for the teacher and others in the classroom to hear, the child will receive the mystery motivator. The class is also told of the expectation that the child ask for the envelope loudly enough that the class can hear.[23][24][25]

Stimulus fadingAfflicted subjects can be brought into a controlled environment with someone with whom they

are at ease and can communicate. Gradually, another person is introduced into the situation. One example of stimulus fading is the sliding-in technique, where a new person is slowly brought into the talking group. This can take a long time for the first one or two faded-in people but may become faster as the patient gets more comfortable with the technique.

As an example, a child may be playing a board game with a family member in a classroom at school. Gradually, the teacher is brought in to play as well. When the child adjusts to the teacher's presence, then a peer is brought in to be a part of the game. Each person is only brought in if the child continues to engage verbally and positively.[23][24][25]

DesensitizationThe subject communicates indirectly with a person to whom he or she is afraid to speak

through such means as email, instant messaging (text, audio, and/or video), online chat, voice or video recordings, and speaking or whispering to an intermediary in the presence of the target person. This can make the subject more comfortable with the idea of communicating with this person.

Shaping[edit]The subject is slowly encouraged to speak. The subject is reinforced first for interacting

nonverbally, then for saying certain sounds (such as the sound that each letter of the alphabet makes) rather than words, then for whispering, and finally saying a word or more.[26]

Spacing[edit]

Spacing is important to integrate, especially with self-modeling. Repeated and spaced out use of interventions is shown to be the most helpful long-term for learning. Viewing videotapes of self-modeling should be shown over a spaced out period of time of approximately 6 weeks.[23][24][25]

Drug treatmentsMany practitioners believe that there is evidence indicating that antidepressants such as SSRIs

may be helpful in treating children and adults with selective mutism and even that medicine is essential to effective treatment.The medication is used to decrease anxiety levels to speed the process of therapy. Use of medication may end after nine to twelve months, once the person has learned skills to cope with anxiety and has become more comfortable in social situations.[27] Medication is more often used for older children, teenagers, and adults whose anxiety has led to depression and other problems.

Medication, when used, should never be considered the entire treatment for a person with selective mutism. While on medication, the person should be in therapy to help them learn how to handle anxiety and prepare them for life without medication.[28]

Anti-depressants have been used in addition to self-modeling and mystery motivation to aid in the learning process.[23][24]

HistoryIn 1877, German physician Adolph Kussmaul described children who were able to speak

normally but often refused to as having a disorder he named aphasia voluntaria.[29] Although this is now an obsolete term, it was part of an early effort to describe the concept now called selective mutism.

In 1980, a study by Torey Hayden identified what she called four "subtypes" of elective mutism, although this set of subtypes is not in current diagnostic use.[30] First, and most common, she described "symbiotic mutism" characterized by a vocal and dominating mother and absent father and the use of mutism as controlling behavior around other adults. Second, the least common, was the "speech phobic mutism" subtype, in which the child showed distinct fear at hearing a recording of their voice. This subtype also involved ritualistic behaviors and was thought to be caused by having been told to keep a family secret.

Hayden's third subtype was "reactive mutism," thought to be caused by trauma or abuse, though not all children put in this category were known to have been abused. These children all showed symptoms of depression and were notably withdrawn, usually showing no facial expressions. In her fourth and last subtype, Hayden described "passive-aggressive mutism" in which silence is used as a display of hostility, connected to antisocial behavior. Some of the children in this group had not been mute until age 9–12. These subtypes are no longer recognized, though "speech phobia" is sometimes used to describe a selectively mute person who appears not to have any symptoms of social anxiety.

The Diagnostic and Statistical Manual of Mental Disorders (DSM), first published in 1952, first included elective mutism in its third edition, published in 1980. Elective mutism was described as "a continuous refusal to speak in almost all social situations" despite normal ability to speak. While "excessive shyness" and other anxiety-related traits were listed as associated features, predisposing factors included "maternal overprotection", mental retardation, and trauma. Elective mutism in the third edition revised (DSM III-R) is described similarly to the third edition except for specifying that the disorder is not related to social phobia.

In 1994, Sue Newman, co-founder of the Selective Mutism Foundation, requested that the fourth edition of the DSM reflect the name change from elective mutism to selective mutism and describe the disorder as a failure to speak. The relation to anxiety disorders was emphasized, particularly in the revised version (DSM IV-TR). As part of the reorganization of the DSM categories,

the DSM-5 moved selective mutism from the section "Disorders Usually First Diagnosed in Infancy, Childhood, or Adolescence" to the section for anxiety disorders.[31]

In popular culturePossibly the most well-known instance of selective (as opposed to total) mutism in popular

culture was depicted by the character of Raj Koothrappali (played by Kunal Nayyar) in the television sitcom The Big Bang Theory. Due to social anxiety, he was unable to speak to women who were not family members, due to fear of rejection. Drinking alcohol suppressed his anxiety, allowing him to speak; however, it negatively affected his personality, making him arrogant, obnoxious, whiny, and perverted. In the episode "The Terminator Decoupling", however, he unknowingly drank alcohol-free beer and due to the placebo effect, was able to hold a normal conversation with actress Summer Glau, who found him interesting and charming. He was later cured in the season six finale "The Bon Voyage Reaction" after his severely socially anxious girlfriend Lucy (played by Kate Micucci) broke up with him.

In Maya Angelou's breakout award winning memoir "I Know Why The Caged Bird Sings", she chronicles her mutism following repeated rapes at age eight by her mother's boyfriend. After the trial he was murdered and Ms. Angelou fell silent for years feeling responsible.

Children's books with a selectively mute protagonist include The Secret Voice of Gina Zhang by Dori Jones Yang and Alvin Ho: Allergic to Girls, School, and Other Scary Things by Lenore Look. Both of these books are set in elementary school and specifically mention selective mutism. In addition, several children's picture books have been written with the specific purpose of educating readers about selective mutism, such as Understanding Katie by selective mutism expert Elisa Shipon-Blum. Adoption Detective by Judith Land mentions selective mutism, extreme shyness, and other social anxiety disorders as evidence of trauma frequently associated with adoption. She explains that adoptees with selective mutism have difficulty verbalizing personal thoughts that are excessively revealing and painful or of a subconscious nature; selective mutism can be highly functional for a child by reducing anxiety and protecting the child from perceived challenges of social interaction. "Speak" by Laurie Halse Anderson includes a main character who doesn't speak after experiencing a rape by a peer at a high school party.

In young adult literature and films, there are several instances of protagonists who do not speak despite having the ability to do so. They usually are mute in all situations, and trauma is a common cause for the mutism, though some make the choice to stop speaking. One well-known book, Cut by Patricia McCormick, features a main character who is entirely silent after facing problems at home and being sent to a psychiatric hospital.

There are various lesser-known books in both young adult and adult fiction, as well as films that follow the same idea. For example, the 2004 made-for-TV movie Samantha: An American Girl Holiday, where one of the three orphans that the protagonist befriended, never said a word for the majority of the story, likely out of emotional trauma due to the death of their parents. In the children's film Jumanji, after the death of their parents the character Peter speaks only to his sister, and only when they are alone.

In the Saturday Night Live Digital Shorts, "Rihanna and Shy Ronnie", and "Ronnie and Clyde", the character "Shy Ronnie" (portrayed by Andy Samberg) cannot rap in front of Rihanna, but starts as soon as she leaves the room, and stops again when she re-enters.

The film Little Voice centers upon a selectively mute singer.The novel The Lock Artist by Steve Hamilton features a teenager Mike (who narrates the

story) suffering from selective mutism after experiencing a traumatic incident in his childhood.In the television show The Fosters, the character Jude Jacob Adams-Foster is diagnosed with

selective mutism briefly in season two when faced with an over amount of stress.

Internet personality and singer Tay Zonday, known for his viral video Chocolate Rain, suffered from anxiety and agoraphobia during his teenage years and was selectively mute during that time.

June and Jennifer Gibbons were known as "The Silent Twins". They were Welsh women of West Indian heritage who stopped speaking except to one another as toddlers. As teenagers, they wrote a series of novels and committed several acts of arson, landing them in Broadmoor Hospital where they came to worldwide attention via interviews and a book by Marjorie Wallace.

Locked-in syndromeLocked-in syndrome (LIS) is a condition in which a patient is aware but cannot move or

communicate verbally due to complete paralysis of nearly all voluntary muscles in the body except for the eyes. Total locked-in syndrome is a version of locked-in syndrome wherein the eyes are paralyzed as well.[1] Fred Plum and Jerome Posner coined the term for this disorder in 1966.[2][3] Locked-in syndrome is also known as cerebromedullospinal disconnection,[4] de-efferented state, pseudocoma,[5] and ventral pontine syndrome.

Unlike persistent vegetative state, in which the upper portions of the brain are damaged and the lower portions are spared, locked-in syndrome is caused by damage to specific portions of the lower brain and brainstem, with no damage to the upper brain.

Possible causes of locked-in syndrome include: Snakebite cases - More frequently from a krait bite and other neurotoxic venoms, as they

cannot, usually, cross the blood–brain barrier Amyotrophic lateral sclerosis (aka Lou Gehrig's disease) Brainstem stroke Diseases of the circulatory system Medication overdose [examples needed], or central pontine myelinolysis secondary to rapid correction

of hyponatremia Multiple sclerosis Damage to nerve cells, particularly destruction of the myelin sheath, caused by disease or

central pontine myelinolysis secondary to rapid correction of hyponatremia [>1 mEq/L/h]) A stroke or brain hemorrhage, usually of the basilar artery Traumatic brain injury

DiagnosisCurare poisoning mimics a total locked-in syndrome by causing paralysis of all voluntarily

controlled skeletal muscles.[8] The respiratory muscles are also paralyzed, but the victim can be kept alive by artificial respiration, such as mouth-to-mouth resuscitation. In a study of 29 army volunteers who were paralyzed with curare, artificial respiration managed to keep an oxygen saturation of always above 85%,[9] a level at which there is no evidence of altered state of consciousness.[10] Spontaneous breathing is resumed after the end of the duration of action of curare, which is generally between 30 minutes[11] and eight hours,[12] depending on the variant of the toxin and dosage.

TreatmentNeither a standard treatment nor a cure is available. Stimulation of muscle reflexes with

electrodes (NMES) has been known to help patients regain some muscle function. Other courses of treatment are often symptomatic.[13] Assistive computer interface technologies, such as Dasher, or OptiKey, combined with eye tracking, may be used to help patients communicate.

PrognosisIt is extremely rare for any significant motor function to return. The majority of locked-in

syndrome patients do not regain motor control, but devices are available to help patients communicate. However, some people with the condition continue to live much longer,[14][15] while in exceptional cases, like that of Kerry Pink[16] and Kate Allatt,[17] a full spontaneous recovery may be achieved.

ResearchNew direct brain interface mechanisms may provide future remedies; one effort in 2002

allowed a fully locked-in patient to answer yes-or-no questions.[18][19][20] Some scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.[21]

Athymhormic syndrome,Athymhormic syndrome, or psychic akinesia, is a rare neurological syndrome characterized

by extreme passivity, apathy, blunted affect, and a profound generalized loss of self-motivation and conscious thought. For example, a patient with this syndrome might sustain severe burns on contact with a hot stove, due to lacking the will to move away despite experiencing severe pain. The existence of such symptoms in patients after damage to certain structures in the brain has been used to support a physical model of motivation in human beings, wherein the limbic loop of the basal ganglia is the initiator of directed action and thought.[1]

The word Athymhormic is derived from the Greek (Greek: Thumos), which means mood or affect, and (Greek: Horme), which means impulse, drive, or appetite. First described by French neurologist Dominique Laplane in 1982 as ""PAP syndrome" (French: perte d'auto-activation psychique, or "loss of psychic autoactivation"), the syndrome is believed to be due to damage to areas of the basal ganglia or frontal cortex, specifically the striatum and globus pallidus, responsible for motivation and executive functions.[2] It may occur without any preexisting psychiatric condition.

SymptomsIt is characterized by an absence of voluntary motion without any apparent motor deficit, and

patients often describe a complete mental void or blank. This is accompanied by reduced affect or emotional concern (athymhormy) and often by compulsions, repetitive actions, or tics. After stimulation from the outside, such as a direct command, the patient is able to move normally and carry out complex physical and mental tasks for as long as they are prompted to continue.

The symptoms may be differentiated from depression because depression requires the existence of sadness or negative thoughts, while athymhormic patients claim to have complete lack of thoughts, positive or negative.

Aboulia or abulia (from the Greek βουλή, meaning "will",[1] with the prefix a- used as a privative), in neurology, refers to a lack of will or initiative and can be seen as a disorder of diminished motivation (DDM). Aboulia falls in the middle of the spectrum of diminished motivation, with apathy being less extreme and akinetic mutism being more extreme than aboulia.[2] A patient with aboulia is unable to act or make decisions independently. It may range in severity from subtle to overwhelming. It is also known as Blocq's disease (which also refers to abasia and astasia-abasia).[3] Abulia was originally considered to be a disorder of the will.[4][5]

Symptoms and signs[edit]Aboulia has been known to clinicians since 1838. However, in the time since its inception, the

definition of Aboulia has been subjected to many different forms, some even contradictory with previous ones.[6] Aboulia has been described as a loss of drive, expression, loss of behavior and speech output, slowing and prolonged speech latency, and reduction of spontaneous thought content and initiative.[7] The clinical features most commonly associated with Aboulia are:[6]

Difficulty in initiating and sustaining purposeful movements Lack of spontaneous movement Reduced spontaneous speech Increased response-time to queries Passivity Reduced emotional responsiveness and spontaneity Reduced social interactions Reduced interest in usual pastimes

Especially in patients with progressive dementia, it may affect feeding.[8] Patients may continue to chew or hold food in their mouths for hours without swallowing it.[8] The behavior may be most evident after these patients have eaten part of their meals and no longer have strong appetites.

Differentiation from other disorders[edit]Both neurologists and psychiatrists recognize Aboulia to be a distinct clinical entity, but its

status as a syndrome is unclear. Although Aboulia has been known to clinicians since 1838, it has been subjected to different interpretations - from 'a pure lack of will', in the absence of motor paralysis to, more recently, being considered 'a reduction in action emotion and cognition'.[6] As a result of the changing definition of Aboulia, there is currently a debate on whether or not Aboulia is a sign or a symptom of another disease, or its own disease that seems to appear in the presence of other more well-researched diseases, such as Alzheimer's disease.[6]

A survey of 2 movement disorder experts, 2 neuropsychiatrists, and 2 rehabilitation experts was conducted and the results did not seem to shed any light on the matter of differentiating aboulia from other DDMs. The experts used the terms apathy and aboulia interchangeably and even debated on whether or not aboulia was a discrete entity, or just a hazy gray area on a spectrum of more defined disorders.[6] Four of the experts said aboulia was a sign and a symptom, but the group was split on whether or not it was a syndrome.[6] Another survey, which consisted of true and false questions about what aboulia is distinct from, whether it is a sign, symptom, or syndrome, where lesions are present in cases of aboulia, what diseases are commonly associated with aboulia, and what current treatments are used for aboulia, was sent to 15 neurologists and 10 psychiatrists. Most experts agreed that aboulia is clinically distinct from depression, akinetic mutism, and alexithymia.[6] However, only 32% believed aboulia was different from apathy, while 44% said they were not different, and 24% were unsure. Yet again, there was disagreement about whether or not aboulia is a sign, symptom, or syndrome.[6][citation

needed]

The study of motivation has been mostly about how stimuli come to acquire significance for animals. Only recently has the study of motivational processes been extended to integrate biological drives and emotional states in the explanation of purposeful behavior in human beings. Considering the number of disorders attributed to a lack of will and motivation, it is essential that aboulia and apathy be defined more precisely to avoid confusion.[6]

Causes[edit]Many different causes of aboulia have been suggested. While there is some debate about the

validity of aboulia as a separate disease, experts mostly agree that aboulia is the result of frontal lesions and not with cerebellar or brainstem lesions.[6] As a result of more and more evidence showing that the mesolimbic and the mesocortical dopamine system are key to motivation and responsiveness to reward, aboulia may be a dopamine-related dysfunction.[7] Aboulia may also result from a variety of brain injuries which cause personality change, such as dementing illnesses, trauma, or intracerebral hemorrhage (stroke), especially stroke causing diffuse injury to the right hemisphere.[9][10]

Damage to the basal ganglia[edit]Injuries to the frontal lobe and/or the basal ganglia can interfere with an individual's ability to

initiate speech, movement, and social interaction. Studies have shown that 5-67% of all patients with traumatic brain injuries and 13% of patients with lesions on their basal ganglia suffer from some form of diminished motivation.[2] Abulia has also been associated with amphetamine withdrawal. It may complicate rehabilitation when a stroke patient is uninterested in performing tasks like walking despite being capable of doing so. It should be differentiated from apraxia, when a brain injured patient has impairment in comprehending the movements necessary to perform a motor task despite not having any paralysis that prevents performing the task; that condition can also result in lack of initiation of activity.

Damage to the capsular genu[edit]

A case study involving two patients who suffered from acute confusional state and aboulia was conducted to see if these symptoms were the result of an infarct in the capsular genu. Using clinical neuropsychological and MRI evaluations at baseline and one year later showed that the cognitive impairment was still there one year after the stroke. Cognitive and behavioral alterations due to a genu infarct are most likely because the thalamo-cortical projection fibers that originate from the ventral-anterior and medial-dorsal nuclei traverse the internal capsule genu. These tracts are part of a complex system of cortical and subcortical frontal circuits through which the flow of information from the entire cortex takes place before reaching the basal ganglia. Cognitive deterioration could have occurred through the genu infarcts affecting the inferior and anterior thalamic peduncles. The interesting thing about this case study was that the patients did not show any functional deficit at the follow-up one year after the stroke and were not depressed but did show diminished motivation. This result supports the idea that aboulia may exist independently of depression as its own syndrome.[11]

Damage to anterior cingulate circuit[edit]The anterior cingulate circuit consists of the anterior cingulate cortex, also referred to as

Brodmann area 24, and its projections to the ventral striatum which includes the ventromedial caudate. The loop continues to connect to the ventral pallidum, which connects to the vental anterior nucleus of the thalamus. This circuit is essential for the initiation of behavior, motivation and goal orientation, which are the very things missing from a patient with a disorder of diminished motivation. Unilateral injury or injury along any point in the circuit leads to aboulia regardless of the side of the injury, but if there is bilateral damage, the patient will exhibit a more extreme case of diminished motivation, akinetic mutism.[9]

Acute caudate vascular lesions[edit]It is well documented that the caudate nucleus is involved in degenerative diseases of the

central nervous system such as Huntington disease. In a case study of 32 acute caudate stroke patients, 48% were found to be experiencing aboulia. Most of the cases where aboulia was present were when the patients had a left caudate infarct that extended into the putamen as seen through a CT or MRI scan.[12]

Diagnosis[edit]Diagnosis for aboulia can be quite difficult because it falls between two other disorders of

diminished motivation, and one could easily see an extreme case of aboulia as akinetic mutism or a lesser case of aboulia as apathy and therefore, not treat the patient appropriately. If it were to be confused with apathy, it might lead to attempts to involve the patient with physical rehabilitation or other interventions where a source of strong motivation would be necessary to succeed but would still be absent. The best way to diagnose aboulia is through clinical observation of the patient as well as questioning of close relatives and loved ones to give the doctor a frame of reference with which they can compare the patient's new behavior to see if there is in fact a case of diminished motivation.[7] In recent years, imaging studies using a CT or MRI scan have been shown to be quite helpful in localizing brain lesions which have been shown to be one of the main causes of aboulia.[6]

Illnesses where aboulia may be present[edit] Depression Schizophrenia Frontotemporal Dementia Parkinson's Disease Huntington's Disease Pick's disease Progressive supranuclear palsy Traumatic Brain Injury Stroke

Alzheimer's disease[edit]A lack of motivation has been reported in 25%-50% of patients with Alzheimer's disease.

While depression is also common in patients with this disease, aboulia is not a mere symptom of depressions because more than half of the patients with Alzheimer's disease with aboulia do not suffer from depression. Several studies have shown that aboulia is most prevalent in cases of severe dementia which may result from reduced metabolic activity in the prefrontal regions of the brain. Patients with Alzheimer's disease and aboulia are significantly older than patients with Alzheimer's who do not lack motivation. Going along with that, the prevalence of aboulia increased from 14% in patients with a mild case Alzheimer's disease to 61% in patients with a severe case of Alzheimer's disease, which most likely developed over time as the patient got older.[8]

Current treatment[edit]Most current treatments for aboulia are pharmacological, including the use of antidepressants.

However, antidepressant treatment is not always successful and have opened up the door for alternative methods of treatment. The first step to successful treatment of aboulia, or any other DDM, is a preliminary evaluation of the patient's general medical condition and fixing the problems that can be fixed easily. This may mean controlling seizures or headaches, arranging physical or cognitive rehabilitation for cognitive and sensorimotor loss, or ensuring optimal hearing, vision, and speech. These elementary steps also increase motivation because improved physical status may enhance functional capacity, drive, and energy and thereby increase the patient's expectation that initiative and effort will be successful.[2]

There are 5 steps to pharmacological treatment:[2]

1. Optimize medical status.2. Diagnose and treat other conditions more specifically associated with diminished motivation

(e.g., apathetic hyperthyroidism, Parkinson's disease).3. Eliminate or reduce doses of psychotropics and other agents that aggravate motivational loss

(e.g., SSRIs, dopamine antagonists).4. Treat depression efficaciously when both DDM and depression are present.5. Increase motivation through use of stimulants, dopamine agonists, or other agents such as

cholinesterase inhibitors.