# Motivation and emotion/Book/2015/Down syndrome and emotion

* Multimedia presentation (3 min)

## Overview

Emotion can be defined as “any mental experience with high intensity and high hedonicity” (Cabanac, 2002). Everyone experiences emotion in day to day life. Whilst the emotional experience of typically functioning individuals in largely studied and in most cases, well understood, the emotional experience of individuals with varying degrees of disability, be it mental or physical is still largely unidentified. This can be due to, in part, the difficulty of identifying and quantifying emotional states in individuals with a diminished cognitive ability. An individual can experience an emotion for either a short or long duration of time, and can be either a positive or negative experience. Emotion can result from a multitude of experiences, including imagination, sensation, memory recall, and assessment (Cabanac, 2002). The use of facial expressions has proven useful in the quantifying of emotional reactions. The study of emotions within the population of individuals with Down syndrome has been in affect since the 1970s, and tools such as the Differential Emotion Scale, Differential Emotions Theory, and the Facial Action Coding System which allows researchers to code and record the emotional expressions of people with Down syndrome and other cognitive or physical syndromes.

## What is Down syndrome?

Down syndrome is one of the most common chromosomal disorders that have been recorded[factual?]. It is estimated that one out of every 900 children born worldwide will be born with Down syndrome[factual?]. People with Down syndrome are usually characterised by their[factual?]:
* Characteristic physical features - including low muscle tone, short stature, and an upward slant to the eyes
* An increased risk of health and developmental challenges - such as congenital heart defects, respiratory problems, sight and hearing deficits, and Thyroid disease
* Intellectual disability - mainly cognitive deficits
In terms of the biological factors behind the diagnoses, those diagnosed with Down syndrome have a total of 47 chromosomes, as opposed to 46. While there are many differerent[spelling?] chromosomal disorders, depending on which chromosome is affected results in differing disorders[grammar?]. People with Down syndrome have an additional chromosome 21, leading to the name Trisomy 21 (Sherman, Allen, Bean & Freeman, 2007).
Down syndrome is one of the most studied (in regards to the biology of the syndrome) genetic syndromes due to its frequency in the population. Studies have shown that the structure of the central nervous system reveals a reduced brainstem size, abnormalities in the development of of the frontal lobes and hippocampus, defective development of the frontal lobes, and diminished size of the limbic area. All of the affected areas are involved in the functioning of the amygdala, which is responsible for the regulation and processing of the emotional information in the brain (de Santana, de Souza & Feitosa, 2014). It has been found that separating cognitive function and emotion cannot occur due to the influence of the frontal regions of the brain and their cross functions[Rewrite to improve clarity] (Hippolyte, Barisnikov & Van Der Linden, 2008)

## Timetable of Emotional Development

Emotion is regulated by many differing systems of the human body and mind, including internal systems (neurophysiological), behavioural components (behavioural and facial reactions), and external components (cultural values, social components, motivation/goals). An individuals[grammar?] ability to regulate their[grammar?] own emotions is developed in childhood, and is dependent (in most cases) on interactions with the social environment surrounding the child (peer interactions, child-caregiver relationships, etc.) (Zeman, Cassano, Perry-Parrish & Stegall, 2006). The ability to identify and understand emotional information while simultaneously managing their own behaviour, develops throughout childhood in the following sequence:

#### Infant development (0-2 years)

Basic emotions such as anger, fear, and joy are displayed within the first 3 months of life, with the child’s emotional development focusing primarily on developing differentiated emotions, with the development of language skills to assist the child in labelling the differing emotional states[factual?]. Infants also develop and engage in social referencing, where the infants use other emotional responses (typically the responses displayed by the primary care-giver) to guide their reactions (Zeman, Cassano, Perry-Parrish & Stegall, 2006).

#### Toddlerhood (1-3 years)

Shame, embarrassment, pride, and other self-conscious emotions are developed during toddlerhood[factual?]. Again, the ability to use language to express emotions enables the toddler to weld-regulate emotion (i.e., they are able to express concerns to an individual who can assist in the regulation, and explanation of their mood) (Zeman, Cassano, Perry-Parrish & Stegall, 2006).

#### Preschool to Early Elementary Age (4-8 years)

The child has developed a large repertoire of behavioural strategies to self-manage their emotions depending on the social context at the time. Also during this period, an increase in understanding of what would be defined as culturally acceptable (depending on the culture) begins to develop and display rules are also developed. Display rules are:
* Neutralisation of emotional expression (the use of a ‘poker face’)
* Amplification of facial expression (e.g. exaggerating pain to gain sympathy from others)
* Substitution of expression (e.g. smiling when the individual is not happy)
* Minimisation of emotion (e.g. looking mildly happy when an individual is ecstatic) (Zeman, Cassano, Perry-Parrish & Stegall, 2006)

#### Middle Childhood (9-11 years)

Children's understanding of the display rules of society increase and being to increase in frequency, and recognise that the emotional reactions displayed by others in a situation may not match their own personal emotional reactions (Zeman, Cassano, Perry-Parrish & Stegall, 2006).

#### Adolescence (12-18 years)

The child’s ability to regulate their[grammar?] emotions increases, and the decision behind the self-regulations becomes more a function of motivation for social factors (Zeman, Cassano, Perry-Parrish & Stegall, 2006).

## Theory of Emotion

There are multiple theories of emotion that have been used throughout the development of the study of motivation and emotion.

#### Differential Emotions Theory (DET)

The Differential Emotions Theory (DET), developed by Carroll Izard, suggests that emotion expressed during infancy is “innate and stereotyped”, and a reliable index of emotion being experienced by the infant, that is, the DET suggests that universally recognisable emotions emerge within the first few months of life. It also suggests that the emotions felt by an infant are “discrete” in the sense that the emotions have characteristic neuromuscular expressive patterns, and distinct subjective qualities. It also suggests that these emotions cluster, or blend together, but only become prominent with development of the infant, but are not dependent on the cognitive development of the infant. Therefore the DET assesses emotions that develop independently (Izard, 2009).

#### Differential Emotions Scale (DES-IV)

The Differential Emotions Scale (4th edition), measures what has been suggested as the 12 fundamental emotions universally seen in the facial expressions of infants. The DES is a standardised instrument that can reliably divide the individuals emotional experience into validated and discrete categories of emotion. The 12 emotions suggested to be universally consistent are: Anger, Contempt, Disgust, Fear, Guilt, Interest, Joy, Sadness, Self-Hostility, Shame, Shyness and Surprise (Bradley & Lang, 1994).

#### Facial Action Coding System (FACS)

Facial Action Coding System (FACS) is a manual developed to code and classify human facial movements. Whilst it was originally developed by a Swedish anatomist, Carl-Herman Hjortsjo, it was adopted by Paul Ekman and Wallace V. Friesen in 1978 where it came into popularity and was further updated in 2002 by Ekman, Friesen, and Joseph C. Hager (Ekman, Friesen & Hager, 2002). Each observable facial movement is labelled an Action Unit (AU), FACS describes the criteria for coding the AUs and the how the AUs may appear in combinations. The FACS manual is designed to be self-instructional. The use of the FACS manual may enable the reader greater awareness of the subtle facial changes that have proven useful for those working in many varying fields. Through the development of FACS it has been shown that from birth, distinctive facial patters for anger, disgust, fear, joy, sadness, and surprise, for both children with Down syndrome as well as typically developing children (Ekman, Friesen & Hager, 2002).

## Emotion in Individuals with Down Syndrome

Down syndrome is the most common cause of mental deficits which is also accompanied by additional deficits in speech, short-term memory development, language production, and auditory senses.
A study conducted by Fernandez-Alcaraz and collages compared a group of individuals with Down syndrome and a group of typically developed individuals. They used four different tasks: A facial discrimination task (participants had to decide whether photographs shown were of the same individual); a facial affect discrimination task (participants had to decide whether the faces showed the same of different emotional expressions); a facial affect naming task (participants had to chose between happiness, anger, sadness, fear or neutral expressions which best fit the expression displayed in the photograph); and the facial affect selection task (participants had to select which of the photographs displayed the emotion indicated by the experimenter). The results of the study showed a significantly poorer result for the individuals with Down syndrome in comparison to the control group. However, individuals with Down syndrome had a higher accuracy when discriminating between individuals[grammar?] faces than among facial expressions, and both individuals with Down syndrome and the control group experienced more accuracy when selecting a facial expression as opposed to selecting a word category (Fernandez-Alcaraz, Extremera, Garcia-Andres & Molina, 2010).
Research has suggested that normally developing children, and children diagnosed with Down syndrome both meet the same developmental milestones, however it has been shown that the children with Down syndrome reach the milestones at a delayed pace. Therefore, many studies assessing such things as smiling and laughter, eye contact, self-recognition, symbolic play, etc, show that when children are match[grammar?] for their cognitive age, rather than their chronological age, they are similar (Zeman, Cassano, Perry-Parrish & Stegall, 2006).
Children and infants with Down syndrome were also found to experience less separation distress,[grammar?] if they do experience separation distress it takes a longer time for the child to become distressed, however they then experience a faster recovery than their normative counterparts. One possible explanation provided for the differences noticed was that the cognitive deficit in the children and infants with Down syndrome impacts their ability to interpret new situations is slowed, which has resulted in slower or less efficient processing of external stimulus (Thompson, Cicchetti, Lamb & Malkin, 1985). Furthermore, the impairment of the cognitive processes can have an effect on the emotional arousal of the child, with the intensity, variability of emotional reactions, and the speed that these reactions (Thompson, Cicchetti, Lamb & Malkin, 1985). Multiple studies have suggested that the facial expressions of children with Down syndrome compared to typically developing children are harder to assess, and that negative emotions in children with Down syndrome are typically presented at a slower rate, and both smiling and laughing present later in age and with a lower intensity, frequency, and duration than typically developing children (Smith & Walden, n.d.). When investigating the use of facial expressions to express emotion in children with Down syndrome, their physical capabilities must be taken into account. The muscle tone of the children with Down syndrome should be taken into account when assassin[spelling?] the results of the studies focusing on the facial expressions of these children. The common occurrence of visual deficits in the children with Down syndrome can also impact on their responses to external stimuli. It has also been found that in children with Down syndrome, primary reflexes (the reflexes developed during the foetal stage of development for the survival of the infant once born i.e. sucking) last longer than their typically developing children. Infants with Down syndrome also have difficulty repressing their emotions once aroused (Hippolyte, Barisnikov & Van Der Linden, 2008).
In a study conducted by Zhang and Cuskelly in 2000, investigating the ability of individuals with Down syndrome to delay gratification, [missing something?] found that children with Down syndrome (with the cognitive age of 4 years) were less efficient at delaying gratification than typically developing children (with the chronological age of 4 years). Cuskelly and collages[spelling?] found indications that persistence was difficult for individuals with Down syndrome to maintain, suggesting that the delay of gratification over an extended period of time may prove to be more difficult for these individuals. However, the results of the study conducted indicated that there were no clear temperamental profiles which can be easily distinguish between individuals with Down syndrome and those of typically developing individuals (Cuskelly & Einam, 2001).
When comparing the temperament of children born prematurely to the temperament of children born with Down Syndrome it was found that the children with Down syndrome displayed less inhibitory control, less sadness, and less attentional focus than the children born prematurely. They also found that children with Down syndrome were more distractible, and had a significantly lower activity level compared to the prematurely born children. It was also found that children with Down syndrome were less likely to complain, and were more accepting of difficulties faced day to day, however, they are more often ill then other children and have more “real life” discomforts than the prematurely born children (Nygaard, Smith & Togersen, 2002).
Studies conducted in the area of emotion and facial expression recognition with individuals diagnosed with Down syndrome have found that children in this category experience difficulties in processing faces and facial expressions, which can lead to implications in relation to perceiving others emotions such as anger, fear and surprise (Fernandez-Alcaraz, Extremera, Garcia-Andres & Molina, 2010). A longitudinal study, covering three years, conducted by Pochon and Declercq in 2013 investigated children with Down syndrome and typically developing children’s ability to recognise the facial expressions of an emotion after hearing a vocalisation. The study provided results that suggested when presented with six facial expressions simultaneously, children with Down syndrome are just as able to identify basic emotion as their typically developing peers. These results suggest that deficits found in other studies should not just be attributed to a cognitive defect, but rather could also be attributed to a difficulty in recognising certain expressions (Pochon & Declercq, 2013).
When presented with emotional stimuli (voices and faces) to children and adults with Down syndrome and typically developing children matched for cognitive age (4 to 6 years), the results showed that the individuals with Down syndrome had more difficulty recognising negative emotion, with specific emphasis on sadness. It was also seen that individuals with Down syndrome, when asked to choose from a selection of preferred emotions (negative and positive) they were more likely to chose a positive expression over a negative (Cuskelly & Einam, 2001).

## Social Implications

It has also been suggested that individuals with Down syndrome undergo changes in the ability to understand emotional states, in themselves and in others. The ability to recognise faces and their emotional expressions play a crucial role in non-verbal communication in humans. Non-verbal communication is used to indicate emotional states and attitudes, and to coordinate conversations, as well as being a precursor of expressive language (de Santana, de Souza & Feitosa, 2014).
In contrast to the studies suggesting that individuals with Down syndrome experience more difficulty recognising negativity, a study conducted by Hippolyte and collages found that participants experienced more difficulty with a neutral and surprised expression. They also found a significant relationship among receptive vocabulary and emotional processing, however there was no relationship with nonverbal reasoning. The results further suggest that individuals with Down syndrome experience deficits in interpersonal relationships, with a main problem being issues with maintaining appropriate interactions. When an individual with Down syndrome is interacting with an individual with a neutral expression, the individual with Down syndrome may not correctly inter prate[spelling?] the others[grammar?] emotional state, as such their reaction may be deemed inappropriate (Hippolyte, Barisnikov & Van Der Linden, 2008).
Due to the physical aspects of Down syndrome, it can be understood as to why individuals with Down syndrome experience some social difficulty if any at all[say what?]. The muscle tone of the individuals with Down syndrome, can in sever[spelling?] cases, prevent the individual being able to adequately display their emotions through facial expressions. The visual and auditory deficits that some of these individuals experience further inhibit their ability to recognise others facial expressions and be able to take social cues and understanding from their peers. Along with the deficit experienced by individuals with Down syndrome in regards to emotion and emotion perception, the stigma associated with individuals with disability, of any kind, also hinders the individuals opportunities to further learn about social and emotional behaviour of others, further hindering the individuals[grammar?] emotional experience.

## Conclusion

Individuals with Down syndrome experience the same developmental milestones as a typically developing individual, just at a delayed pace. It is difficult to say where each individual child is developmentally at any given time as just like typically developing children; children with Down syndrome develop at their own pace. Regardless of the pace, children with Down syndrome reach the emotional developmental milestones of typically developing children, the milestones must be reached in order for these children to achieve functional relationships with others, as well as being able to understand what they themselves are feeling and being able to interpret what it means[Rewrite to improve clarity].
The cognitive deficits that these individuals[grammar?] experience could very possibly be the reason that these individuals have a slightly delayed emotional development. A large number of studies conducted over the years have provided evidence that individuals with Down syndrome have more difficulty recognising negative emotion in others, while they themselves experience less negative emotion (when in a strange situation) than their typically developing peers. This has only been possible since the development of the various tools and measures introduced to quantify and record innate facial expressions of anyone.
It has also been shown that when given the choice between a positive emotion (joy) and a negative emotion (anger) the individual will most likely chose[spelling?] the positive emotion over the negative[factual?], this may be due to their deficit in distinguishing negative emotions. However, the literature does not completely agree with the reasoning as to why these individuals experience a different emotional life compared to typical individuals. Further research should be encouraged to further investigate, and further research into the emotional experience of adults with Down syndrome should also be an area of interest[vague].
Though individuals with Down syndrome lead a somewhat different life to those of their typically developed peers, they are still able to lead long[factual?], healthy and fulfilled lives. The understanding of their emotional experience is still somewhat of a mystery, and thus it is difficult to assess the quality of their emotional experience in day to day life and indeed across the lifespan. However, while the [which?] studies suggest that these individuals have difficulty ascertaining the emotions felt by others, most studies agree that these individuals experience an overall happy and content life.