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Adaptive and Maladaptive Behaviour in Adults with Fragile X Syndrome and Down Syndrome

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Title: Adaptive and Maladaptive Behaviour in Adults with Fragile X Syndrome and Down Syndrome
Language: English
Authors: Begoña Medina-Gómez (ORCID 0000-0002-4772-7798), Gloria Pérez de Albéniz-Garrote (ORCID 0000-0002-7776-6640)
Source: International Journal of Disability, Development and Education. 2025 72(6):1121-1133.
Availability: Routledge. Available from: Taylor & Francis, Ltd. 530 Walnut Street Suite 850, Philadelphia, PA 19106. Tel: 800-354-1420; Tel: 215-625-8900; Fax: 215-207-0050; Web site: http://www.tandf.co.uk/journals
Peer Reviewed: Y
Page Count: 13
Publication Date: 2025
Document Type: Journal Articles
Reports - Research
Descriptors: Behavior Problems, Adults, Down Syndrome, Genetic Disorders, Foreign Countries, Daily Living Skills, Interpersonal Relationship, Social Behavior, Antisocial Behavior, Correlation
Geographic Terms: Spain
Assessment and Survey Identifiers: Adaptive Behavior Scale
DOI: 10.1080/1034912X.2024.2427616
ISSN: 1034-912X
1465-346X
Abstract: This study assesses adaptive and maladaptive behaviour in a sample of 68 adults. The Spanish version of the Adaptive Behaviour Scale: Residence and Community (ABS-RC:2) was applied to 30 subjects with Fragile X Syndrome (FXS) and 38 subjects with Down Syndrome (DS) aged between 25 and 57. The results show that adults with FXS score higher than do those with DS in some adaptive behaviours, such as physical development and economic activities while scoring less than adults with DS in self-direction. Adults with FXS show more stereotyped behaviours, hyperactivity, and social isolation, but less trustworthiness and disturbing interpersonal behaviours than do those with DS. A relation was found between both behaviours in DS: the higher the scores in maladaptive behaviour, the lower those in adaptive behaviour. However, no such relation was found in individuals with FXS.
Abstractor: As Provided
Entry Date: 2025
Accession Number: EJ1490777
Database: ERIC
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  Value: <anid>AN0187437675;54q01sep.25;2025Aug22.05:48;v2.2.500</anid> <title id="AN0187437675-1">Adaptive and Maladaptive Behaviour in Adults with Fragile X Syndrome and Down Syndrome </title> <p>This study assesses adaptive and maladaptive behaviour in a sample of 68 adults. The Spanish version of the Adaptive Behaviour Scale: Residence and Community (ABS-RC:2) was applied to 30 subjects with Fragile X Syndrome (FXS) and 38 subjects with Down Syndrome (DS) aged between 25 and 57. The results show that adults with FXS score higher than do those with DS in some adaptive behaviours, such as physical development and economic activities while scoring less than adults with DS in self-direction. Adults with FXS show more stereotyped behaviours, hyperactivity, and social isolation, but less trustworthiness and disturbing interpersonal behaviours than do those with DS. A relation was found between both behaviours in DS: the higher the scores in maladaptive behaviour, the lower those in adaptive behaviour. However, no such relation was found in individuals with FXS.</p> <p>Keywords: Adaptive behaviour; down syndrome; fragile X syndrome; maladaptive behaviour</p> <hd id="AN0187437675-2">Introduction</hd> <p>Intellectual disability is characterised by a significant impairment of intellectual functioning and in adaptive behaviour, as has been evidenced in conceptual, social, and practical adaptive skills. This disability, which originates before 22 years old (Schalock et al., [<reflink idref="bib40" id="ref1">40</reflink>]) can be due to different causes: 30–50% of the cases are associated with different genetic disorders, among which the fragile X syndrome and the Down syndrome can be highlighted due to its incidence (Moskowitiz & Jones, [<reflink idref="bib34" id="ref2">34</reflink>]). A study in the matter carried out in Europe shows that fragile X syndrome has an incidence of 1.4 in males and 0.9 in females out of 10,000 inhabitants (Reches, [<reflink idref="bib37" id="ref3">37</reflink>]). On the other hand, Down syndrome had an incidence of 7.4 out of 10,000 inhabitants in Europe in 2015 (De Graaf et al., [<reflink idref="bib19" id="ref4">19</reflink>]).</p> <p>Fragile X syndrome is currently the main hereditary cause of intellectual disability, provoked by Cytosine-Guanine-Guanine repeat expansion in the FMR1 gene, placed in the long arm of the X chromosome (Hagerman & Hagerman, [<reflink idref="bib24" id="ref5">24</reflink>]). Repetitions exceed 200, potentially achieving 2,000 in people with the full mutation, while this number varies between 50 and 200 in individuals with premutation (J. A. Artigas, [<reflink idref="bib2" id="ref6">2</reflink>]). The mutation causes FMR1 protein deficiency (Fragile X Syndrome Mental Retardation Protein), which seems to be responsible for cognitive disorders and physical traits and, besides, is considered to be a potential predictor of behavioural characteristics in this group (Bartholomay et al., [<reflink idref="bib5" id="ref7">5</reflink>]). The behavioural phenotype is mainly characterised by social anxiety (Cordeiro et al., [<reflink idref="bib15" id="ref8">15</reflink>]; Schalock et al., [<reflink idref="bib39" id="ref9">39</reflink>]), sensory hypersensitivity (I. L. Cohen et al., [<reflink idref="bib13" id="ref10">13</reflink>]), language difficulties (Brady et al., [<reflink idref="bib7" id="ref11">7</reflink>]; Raspa et al., [<reflink idref="bib36" id="ref12">36</reflink>]), self-destructive, stereotyped, and aggressive behaviours, hyperactivity and impulsiveness (Cornish et al., [<reflink idref="bib17" id="ref13">17</reflink>]). J. Artigas and Brun ([<reflink idref="bib1" id="ref14">1</reflink>]) found that fragile X syndrome behavioural characteristics are linked to genetic disorders and, thus, do not occur as a result of intellectual disability.</p> <p>As to Down syndrome, it is the most common genetic cause of intellectual disability. It is mostly provoked by the growth of an extra chromosome in the 21st pair, which causes serious alterations in the expression of several genes (Huete, [<reflink idref="bib28" id="ref15">28</reflink>]). Concerning the phenotype, more than 100 features have been described, there being a high variability among people with this syndrome. The characteristic that most of these people have in common is intellectual disability, but also a wide range of psychomotor and communicative features can be observed (motor planning, receptive language, ...) (Brady et al., [<reflink idref="bib7" id="ref16">7</reflink>]; Raspa et al., [<reflink idref="bib36" id="ref17">36</reflink>]).</p> <p>As has been verified, fragile X syndrome and Down syndrome are two of the main syndromes associated with intellectual disability. This disability inevitably affects independent functioning. However, current studies reveal that IQ is not the only feature which determines adult functioning, since, adaptive behaviour has an even more relevant role in this. Adaptive behaviour is a multidimensional construct understood as a set of conceptual, social, and practical skills learnt in order to function in daily living (Schalock et al., [<reflink idref="bib39" id="ref18">39</reflink>]).</p> <p>People with intellectual disability show limitations in their adaptive behaviour that affect individual functioning and are always visible (Chorpita, [<reflink idref="bib12" id="ref19">12</reflink>]). These limitations are part of the criteria set for the diagnosis, classification, and deployment of the required support (Schalock et al., [<reflink idref="bib39" id="ref20">39</reflink>]). Therefore, adaptive behaviour is an important concept in the evaluation, treatment, and deployment of supports for people with fragile X syndrome or Down syndrome.</p> <p>The few studies that have analysed adaptive behaviour in both syndromes reveal socialisation scores to be lower in fragile X syndrome than in Down syndrome, as individuals with fragile X syndrome see themselves more limited when it comes to achieving adequate social adaptation due to specific phenotypic characteristics such as hyperactivity, isolation, or poor social functioning (Medina-Gómez, [<reflink idref="bib30" id="ref21">30</reflink>]). Zhu et al. ([<reflink idref="bib44" id="ref22">44</reflink>]) have also found that children with fragile X syndrome attain lower scores in socialisation, prevocational-vocational training, and self-direction activities than those with Down syndrome. However, individuals with fragile X syndrome have better self-management skills than those with Down syndrome (Burack et al., [<reflink idref="bib9" id="ref23">9</reflink>]).</p> <p>Currently, new studies are providing good examples of how genetic variables can be an important risk factor for the development of maladaptive behaviour in people with intellectual disability. In fact, many of these behavioural changes are considered part of the behavioural phenotype of fragile X syndrome and Down syndrome (Hustyi et al., [<reflink idref="bib29" id="ref24">29</reflink>]; Smith et al., [<reflink idref="bib41" id="ref25">41</reflink>]; Wheeler et al., [<reflink idref="bib43" id="ref26">43</reflink>]). However, the presence of maladaptive behaviour is not relevant to diagnosis, since just a few individuals show it. The most usual maladaptive behaviours seen in studies carried out with children with fragile X syndrome are self-destructive (hand biting, hitting, pinching the skin), stereotyped, hyperactive, and aggressive behaviours (Esbensen et al., [<reflink idref="bib23" id="ref27">23</reflink>]). Studies about maladaptive behaviour in people with Down syndrome do not provide information that is coherent enough; some indicate that this group presents more maladaptive behaviours than in other causes of intellectual disability. Other studies, however, find no higher prevalence of these maladaptive behaviours. Thus, people with Down syndrome usually present less maladaptive behaviour, with challenging, hyperactive, and aggressive behaviour being the most frequently observed ones (J. A. Artigas, [<reflink idref="bib3" id="ref28">3</reflink>]; Ersoy et al., [<reflink idref="bib22" id="ref29">22</reflink>]). None of the examined articles contains data about the occurrence of maladaptive behaviour in fragile X syndrome or Down syndrome, but there are studies that deal with the incidence of psychiatric disorders (Cordeiro et al., [<reflink idref="bib16" id="ref30">16</reflink>]).</p> <p>Regarding the relationship between adaptive and maladaptive behaviour, the studies examined do not lead to any conclusive results. According to some authors, maladaptive behaviour often interferes with the acquisition or performance of adaptive skills, which sometimes restricts access to community environments (Medina-Gómez et al., [<reflink idref="bib32" id="ref31">32</reflink>]). Other authors state that high levels of adaptive behaviours may help, at least party, prevent exponential increases in maladaptive behaviours (Hustyi et al., [<reflink idref="bib29" id="ref32">29</reflink>]).</p> <p>On the contrary, a study conducted on children with fragile X syndrome showed a significative relationship between both behaviours (Côté et al., [<reflink idref="bib18" id="ref33">18</reflink>]). Another study shows very low correlations between these two behaviours, which tend to be higher in people with more severe degrees of disability (Harrison, [<reflink idref="bib26" id="ref34">26</reflink>]). These studies imply that the lack of coincidence could be due to the comparison between participants with different IQ levels or to the way to assess adaptive behaviour, which can depend on a wide range of personal and environmental factors. Currently, most studies carried out in Spain on adaptive and maladaptive behaviour in people with fragile X syndrome or Down syndrome have mainly addressed children and not adults.</p> <p>Furthermore, there are studies indicating that maladaptive behaviour of children with fragile X syndrome and Down syndrome is often the parents' major concern and it is associated, in many cases, to the parents' high levels of stress, depression, and a poor living environment (Hartley et al., [<reflink idref="bib27" id="ref35">27</reflink>]; Smith et al., [<reflink idref="bib41" id="ref36">41</reflink>]; Wheeler et al., [<reflink idref="bib43" id="ref37">43</reflink>]).</p> <p>More research, though, is required in the analysis of any connections between genetic disorders and behaviour in adults with intellectual disability to gain more insight as to how behavioural phenotypes develop. According to different authors (Artigas & Brun, [<reflink idref="bib1" id="ref38">1</reflink>]; Edgin, [<reflink idref="bib21" id="ref39">21</reflink>]) people with fragile X syndrome and Down syndrome show a behavioural phenotype which identifies them and makes them different from other adults with intellectual disability (even when there is intersubject variability). Therefore, knowing such differences can help find intervention methods more adapted to each individual's needs. In other words, certain behavioural limitations appreciated in people with specific genetic disorders can be substantially improved when implementing the adequate interventions, as adaptive behaviour can be learnt if the necessary opportunities are provided. As far as we know, some studies in this respect have been conducted on children and youngsters (Bargagna et al., [<reflink idref="bib4" id="ref40">4</reflink>]; Côté et al., [<reflink idref="bib18" id="ref41">18</reflink>]; Zhu et al., [<reflink idref="bib44" id="ref42">44</reflink>]) but not on adults. Therefore, the research question would be what is the level in adaptive and maladaptive behaviour, and is there a relationship between the two as a function of the genetic syndrome? The objectives are: 1) to analyse possible variations in the level or pattern of different domains adaptive behaviour and maladaptive behaviour among adults with fragile X syndrome or Down syndrome, 2) the examine the possible relationship between adaptive and maladaptive behaviours in both groups.</p> <hd id="AN0187437675-3">Materials and Methods</hd> <p></p> <hd id="AN0187437675-4">Participants</hd> <p>The participants of this study were 68 male adults aged 25–45 (<emph>M</emph> = 34.49, SD = 5.63), 30 of them with fragile X syndrome (<emph>M</emph> = 33.20, SD = 5.94) and 38 with Down syndrome [<emph>M</emph> = 35.50, SD = 5.24, <emph>t</emph> = 1.69; <emph>p</emph> =.95, df = 66 (−5.01,.41)]. Forming the fragile X syndrome group required genetic confirmation of the full mutation by means of the polymerase chain reaction molecular technique. As to Down syndrome, genetic confirmation of the diagnosis was required.</p> <p>All Down syndrome and fragile X syndrome attended care institutions for people with intellectual and development disabilities in Burgos (Spain) out of the premature ageing scheme. The sample was made up of all of the individuals attending these care centres who accepted to participate and who presented one of the syndromes. Another requirement to participate was to present a moderate IQ level (55–40), according to DSM-V criteria, to ensure that any possible differences in adaptive behaviour would not happen as a result of IQ level, since some authors indicate that the higher the IQ level is, the higher scores in adaptive behaviour are attained (Carrillo, [<reflink idref="bib11" id="ref43">11</reflink>]; Medina-Gómez et al., [<reflink idref="bib32" id="ref44">32</reflink>]). The purpose was for no significant differences to be found between the two independent groups (Studen's <emph>t</emph>), while assuming that variance would be the same (IQ fragile X syndrome, <emph>M</emph> = 34.66, SD = 5.48; IQ Down syndrome, <emph>M</emph> = 34.86, SD = 4.44, <emph>t</emph> =.17, <emph>p</emph> =.86, df = 66 (−2.6,2.2). Females were excluded from this study because fragile X syndrome is linked to the X chromosome and the phenotypic features are different from one sex to the other. Also, the study excluded males with any other psychopathologic diagnosis besides intellectual disability (i.e. autism, Alzheimer, anxiety disorder, depression) and those who took psychiatric medication. Table 1 includes some of the sociodemographic data of each group, as obtained from the individual records, where it was also possible to check recent clinical history and the IQ level, obtained through the certificate of disability issued by the management of social services of the regional government (Junta de Castilla y León) were also checked.</p> <p>Table 1. Demographic characteristics of the normative sample.</p> <p> <ephtml> <table><thead><tr><td /><td>Fragile X syndrome (<italic>n</italic> = 30)</td><td>Down syndrome (<italic>n</italic> = 38)</td></tr><tr><td>Characteristics</td><td><italic>n</italic></td><td><italic>%</italic></td><td><italic>n</italic></td><td><italic>%</italic></td></tr></thead><tbody><tr><td>Age</td><td /><td /><td /><td /></tr><tr><td><30 31-40 >41</td><td>12 14 4</td><td>40.0 46.7 13.3</td><td>7 22 9</td><td>18.4 57.9 23.7</td></tr><tr><td>Socioeconomic Level</td><td /><td /><td /><td /></tr><tr><td>Low Medium High</td><td>6 19 5</td><td>20.0 63.3 16.7</td><td>6 27 5</td><td>15.8 71,7 13.2</td></tr><tr><td>Place of Residence</td><td /><td /><td /><td /></tr><tr><td>With family Institution</td><td>27 3</td><td>90.0 10.0</td><td>28 10</td><td>73.7 26.3</td></tr></tbody></table> </ephtml> </p> <hd id="AN0187437675-5">Instrument</hd> <p>Adaptive Behavior Scale-Residential and Community: Second Edition (ABS-RC:2; Nihira et al., [<reflink idref="bib35" id="ref45">35</reflink>]) was used, as it was one of the few available scales in Spanish language to assess adaptive behaviour when the study was carried out. This scale can be applied to adults with intellectual disability until the age of 69. It has two parts: the first one assesses adaptive behaviour with 73 items grouped into 10 domains, while the second assesses maladaptive behaviour with 41 items grouped into eight domains (Table 2).</p> <p>Table 2. ABS-RC: 2, domains and subdomains.</p> <p> <ephtml> <table><tbody><tr><td><bold>Domain and subdomains. Part One</bold></td></tr><tr><td>Independent functioning: Eating, toilet use, cleanliness, appearance, care of clothing, dressing and undressing, travel and other independent functioning. Physical development: Sensory development and motor development Economic activity: Money handing and budgeting, and shopping skills. Language development: Expression, verbal comprehension, social language development. Number and time: Numbers, time and time concept. Domestic activity: Cleaning, kitchen and other domestic duties. Prevocational/vocational activity: Job complexity, work/school-job performance. Self-direction: Initiative, perseverance, leisure time. Responsibility: Personal belongings, general responsibility, personal responsibility. Socialization: Cooperation, consideration for others, awareness of others, interaction with others, participation in group activities.</td></tr><tr><td><bold>Domain and subdomains. Part Two</bold></td></tr><tr><td>Social behaviour: Threatens or does physical violence, has violent temper or temper tantrums, teases or gossips about others, bosses and manipulates others, uses angry language, reacts poorly to frustration and disrupts others´activities. Conformity: Ignores regulations or regular routines, resists following instructions or orders, has imprudent or rebellious attitude towards authority; is absent from the proper assignments or places, run away or attempts to run away and misbehaves in group setting. Trustworthiness: Shows disrespect for others´property, takes others´property without permission, lies or cheats, damages personal property, damages public property and damages others´property. Stereotyped behaviour and hyperactive: Has stereotyped behaviours, has inappropriate interpersonal manners, has disturbing vocal or speech habits, has unacceptable oral habits, has hyperactive tendencies. Sexual behaviour: Removes or tears off own clothing, engages in inappropriate masturbation, exposes body improperly and has sexual behaviour that is sociality unacceptable. Self-abusive behaviour: Has other eccentric habits and tendencies, does physical violence to self and has strange and unacceptable habits. Social isolation: Is inactive, is withdrawn, is shy and has peculiar posture or odd mannerisms. Disturbing interpersonal behaviour: Tends to overestimate own abilities, reacts poorly to criticism, demands excessive, attention or praise, seems to feel persecuted, has hypochondriacal tendencies and has other signs of emotional instabilities.</td></tr></tbody></table> </ephtml> </p> <p>Scale items are rated in two ways: in some of them, only the highest behaviour level shown by the person is selected, while others indicate whether or not the individual is able to perform the behaviour in order to make the total sum of each domain or subdomain. According to the authors of the original version (Nihira et al., [<reflink idref="bib35" id="ref46">35</reflink>]), the scale offers five types of scores, with direct scoring being the most frequent for research purposes.</p> <p>In a previous study, Medina-Gómez et al. ([<reflink idref="bib32" id="ref47">32</reflink>]) proved that the translated and adapted Spanish version of the ABS-RC:2 has an excellent internal consistency in each of its constituent parts (Cronbach's alpha): adaptive behaviour (α=.91) and maladaptive behaviour (α=.87), reliability coefficient and test–retest (α=.88 and α =.94, respectively), and inter-rater reliability (α=.96 and α =.87). It correlates positively with the criteria of the equivalent categories in adaptive behaviour and behaviour problems of the Inventory for Client and Agency Planning (ICAP) (Bruininks et al., [<reflink idref="bib8" id="ref48">8</reflink>]), adapted and translated into Spanish by Montero ([<reflink idref="bib33" id="ref49">33</reflink>]).</p> <hd id="AN0187437675-6">Procedure</hd> <p>This observational, descriptive, descriptive, cross-sectional and correlational study was carried out for 2 years. First of all, the institutions to which the participants belonged were informed of this research and requested authorisation. The psychologists who worked in these institutions provided data about the participants´ clinical history and they helped to make a first selection of those participants who met the admission criteria. Subsequently, the inclusion criteria were presented, and relatives were invited to attend an information meeting where they were asked to sign an informed consent form to take part in the study. All voluntarily agreed to participate in the study (<emph>n</emph> = 68). Before applying the scale, once the sample had been drawn, testers (two psychologists who worked daily with the participants, with more than 10 years' experience and acquainted with the participants) completed the ABS-RC:2, for which they had been duly trained, the estimated time to perform the evaluation of each user ranged from 30 to 60 minutes.</p> <p>Once the test had been completed, the data obtained were processed using SPSS-25 Statistical Package. The scores obtained in the items of every domain were added to the total scores in adaptive and maladaptive behaviours to check whether there were significant differences between score averages. Seeing that samples were not normally distributed and that the variables were quantitative discrete, Mann–Withey U Test was deployed for independent samples (<emph>p</emph> <.05). According to the recommendations of Wassertein and Lazar ([<reflink idref="bib42" id="ref50">42</reflink>]), the effect size was measured using Rosenthal's r and its interpretation followed Cohen's suggestions (Cohen, [<reflink idref="bib14" id="ref51">14</reflink>]). In order to determine whether there was a relationship between adaptive and maladaptive behaviour, the correlations (Pearson's r) between the total sums obtained in each group (<emph>p</emph> <.01) were considered. As to scale reliability, we obtained Cronbach's alpha values of.84 and.52, respectively.</p> <hd id="AN0187437675-7">Results</hd> <p>Regarding the first objective, which aims to analyse the differences between adaptive and maladaptive patterns of behaviour adults with fragile X syndrome obtained higher scores in six out of the 10 domains of adaptive behaviour: independent functioning, physical development, economic activity, number and time, prevocational-vocational activity, socialisation, and total sum (Table 2). Nevertheless, only in physical development and economic activity were there remarkable differences. Adults with Down syndrome scored higher in language development, domestic activity, responsibility, and self-direction but differences were only significant in this last case (<emph>p</emph> <.05). Effect size could be considered medium to low in physical development and economic activity, which means that the average score in the fragile X syndrome group shows an approximate standard deviation of 0.3 with respect to the Down syndrome group. As for self-direction, lower differences have been found between the average score of one group and the other (.2).</p> <p>Data extracted from the analysis of maladaptive behaviour demonstrate that adults with fragile X syndrome scored significantly higher in stereotyped behaviour and hyperactivity, social isolation, and the total maladaptive behaviour sum, observing that the effect size is close to.5. By contrast, adults with Down syndrome obtained higher scores in the remaining domains, being the difference only significant in trustworthiness. In this case, the effect size was medium to low (.3). In both fragile X syndrome and Down syndrome groups, it can be observed that either they do not score at all or they obtain a low score (Table 3).</p> <p>Table 3. Average comparison between domains in adaptive and maladaptive behaviour (Mann–Whitney U).</p> <p> <ephtml> <table><thead><tr><td /><td>Fragile X syndrome</td><td>Down syndrome</td><td /><td /><td /><td /><td /></tr></thead><tbody><tr><td>Domain</td><td><italic>M(DS)</italic></td><td><italic>M(DS)</italic></td><td><italic>U</italic></td><td>W</td><td><italic>Z</italic></td><td><italic>Sig</italic></td><td><italic>d</italic></td></tr><tr><td>IF</td><td>84.47(10.67)</td><td>81.00(13.10)</td><td>464.00</td><td>1205.00</td><td>−1.31</td><td>.19</td><td>−.16</td></tr><tr><td>PD</td><td>23.00(1.23)</td><td>21.84(1.69)</td><td>354.00</td><td>1095.00</td><td>−2.75</td><td>.01</td><td>−.33</td></tr><tr><td>EA</td><td>6.07(2.05)</td><td>4.95(4.33)</td><td>334.00</td><td>1075.00</td><td>−2.94</td><td>.00</td><td>−.36</td></tr><tr><td>LD</td><td>21.80(5.10)</td><td>22.26(6.31)</td><td>500.00</td><td>1241.00</td><td>−.87</td><td>.38</td><td>−.10</td></tr><tr><td>NT</td><td>6.67(3.20)</td><td>5.42(3.15)</td><td>420.00</td><td>1161.00</td><td>−1.87</td><td>.06</td><td>−.23</td></tr><tr><td>DA</td><td>6.20(1.82)</td><td>7.26(2.76)</td><td>470.00</td><td>935.00</td><td>−1.25</td><td>.21</td><td>−.15</td></tr><tr><td>PVA</td><td>8.53(1.81)</td><td>8.42(1.92)</td><td>558.00</td><td>1299.00</td><td>−.15</td><td>.88</td><td>−.02</td></tr><tr><td>SED</td><td>14.53(3.10)</td><td>16.26(3.34)</td><td>398.00</td><td>863.00</td><td>−2.14</td><td>.03</td><td>−.26</td></tr><tr><td>RE</td><td>7.20(1.29)</td><td>7.26(1.44)</td><td>568.00</td><td>1033.00</td><td>−.02</td><td>.98</td><td>−.00</td></tr><tr><td>SO</td><td>18.60(2.25)</td><td>18.53(2.85)</td><td>520.00</td><td>1261.00</td><td>−.62</td><td>.53</td><td>−.07</td></tr><tr><td>SCA</td><td>197.07(27.40)</td><td>193.21(37.41)</td><td>466.00</td><td>1207.00</td><td>−1.28</td><td>.20</td><td>−.15</td></tr><tr><td>SB</td><td>1.67(2.96)</td><td>1.37(1.97)</td><td>534.00</td><td>999.00</td><td>−.48</td><td>.63</td><td>−.06</td></tr><tr><td>CO</td><td>1.13(1.38)</td><td>1.53(2.81)</td><td>514.00</td><td>1255.00</td><td>−.76</td><td>.44</td><td>−.09</td></tr><tr><td>TR</td><td>0.00(0.00)</td><td>0.42(0.89)</td><td>450.00</td><td>915.00</td><td>−2.65</td><td>.01</td><td>−.32</td></tr><tr><td>SHB</td><td>3.33(3.03)</td><td>0.84(1.68)</td><td>270.00</td><td>1011.00</td><td>−4.02</td><td>.00</td><td>−.49</td></tr><tr><td>SXB</td><td>0.00(0.00)</td><td>0.05(0.23)</td><td>540.00</td><td>1005.00</td><td>−1.27</td><td>.20</td><td>−.15</td></tr><tr><td>SAB</td><td>0.20(0.40)</td><td>0.42(0.89)</td><td>546.00</td><td>1011.00</td><td>−.42</td><td>.67</td><td>−.05</td></tr><tr><td>SI</td><td>1.80(1.70)</td><td>0.42(0.68)</td><td>264.00</td><td>1005.00</td><td>−4.07</td><td>.00</td><td>−.49</td></tr><tr><td>DIB</td><td>0.67(0.48)</td><td>1.32(1.89)</td><td>560.00</td><td>1025.00</td><td>−.13</td><td>.89</td><td>−.02</td></tr><tr><td>SCD</td><td>8.80(4.08)</td><td>6.37(6.92)</td><td>374.00</td><td>1115.00</td><td>−2.43</td><td>.01</td><td>−.29</td></tr></tbody></table> </ephtml> </p> <p>1 Note: D (domains), FXS (Fragile X Syndrome X), DS (Down Syndrome), IF (independent functioning), PD (physical development), EA (economic activity), LD (language development), NT (numbers-time), DA (domestic activity), PVA (prevocational- vocational activity), SED (self-direction), RE (responsibility), SO (socialisation), SCA (adaptive behaviour total sum) SB (social behaviour), CO (conformity), TR (trustworthiness), SHB (stereotyped behaviour and hyperactive), SXB (sexual behaviour), SAB (self-abusive behaviour), SI (social isolation), DIB (disrupting interpersonal behaviour), SCD (maladaptive behaviour total sum), U (Mann-Whitney U), W (Wilcoxson), <emph>d</emph> (effect size).</p> <p>Regarding the second objective, correlations (Pearson's <emph>r</emph>) were analysed between adaptive and maladaptive behaviour total sums in each group (Table 4).</p> <p>Table 4. Correlations between adaptive and maladaptive behaviour in both groups.</p> <p> <ephtml> <table><thead><tr><td /><td>Fragile X Syndrome</td><td>Down Syndrome</td></tr></thead><tbody><tr><td /><td>Adaptive behaviour sum</td><td>Maladaptive behaviour sum</td></tr><tr><td>Adaptive behaviour sum</td><td><italic>r</italic></td><td>.15</td><td>−.42**</td></tr><tr><td>Sig. (bilateral)</td><td>.42</td><td>.01</td></tr></tbody></table> </ephtml> </p> <p>2 **Correlation is significant at 0.01 level (bilateral).</p> <p>Results show that, in the particular case of fragile X syndrome, there is no correlation between behaviours, but, when dealing with Down syndrome, there is a moderate negative correlation (<emph>p</emph> <.05). After performing linear regression on Down syndrome, 18% of variance in adaptive behaviour is explained by maladaptive behaviour (<emph>t</emph> = −2.82, df = 37, <emph>p</emph> <.01).</p> <hd id="AN0187437675-8">Discussion</hd> <p>The aim of this study was, on the one hand, to analyse differences between adaptive and maladaptive behaviour in adults with fragile X syndrome or Down syndrome and, on the other hand, to analyse the possible relation between both behaviours in both groups. Results show that people with fragile X syndrome score significantly higher in physical development and economic activity of adaptive behaviour, while people with Down syndrome scored higher in self-direction, even when the effect size was not high. According to some authors (Medina-Gómez et al., [<reflink idref="bib32" id="ref52">32</reflink>]; Nihira et al., [<reflink idref="bib35" id="ref53">35</reflink>]), adaptive behaviour domains are typically acquired before the age of 16. In this study, the results from the analysis of adaptive behaviour in adults with fragile X syndrome or Down syndrome suggest that participants present limitations in almost all domains, as they do not obtain the highest score. Therefore, no major differences have been found between fragile X syndrome and Down syndrome adults as to adaptive behaviour, except in physical development and self-direction.</p> <p>Contrary to previous studies which indicate that people with fragile X syndrome work more independently and have fewer social skills than people with Down syndrome, this study does not prove any significative differences in these domains (Bargagna et al., [<reflink idref="bib4" id="ref54">4</reflink>]). This could be explained by the fact that the sample of adults analysed have had the chance of gaining further skills throughout their lives. According to Husty et al. ([<reflink idref="bib29" id="ref55">29</reflink>]), adaptive behaviour increases with age.</p> <p>There is an increasing number of authors who consider that the physical development domain has to be removed from the directory of adaptive behaviour (Harries et al., [<reflink idref="bib25" id="ref56">25</reflink>]), since it makes reference to sensory and motor skills which cannot be learnt throughout the development. In fact, most adults with fragile X syndrome show good physical development and, although some people with Down syndrome present limitations in this respect, such limitations can be compensated with adequate social-health care and disability aids. Thus, for example, 50% of children with Down syndrome have conductive, sensorineural or mixed hearing loss highly associated with respiratory infections and higher earwax viscosity, while 50% of children with fragile X syndrome can present repeated otitis in the first year with no consequences in adulthood. Also, children with Down syndrome can suffer around 70% of eyesight refractory issues, while X fragile syndrome only 20% (Calvo et al., [<reflink idref="bib10" id="ref57">10</reflink>]).</p> <p>Findings in this study are consistent with those put forward by Zhu et al. ([<reflink idref="bib44" id="ref58">44</reflink>]) when suggesting that adults with Down syndrome have more initiative, are more active, pay more attention, and are more persistent (self-direction) than adults with fragile X syndrome. This could be due to difficulties that people with fragile X syndrome show when giving socially accepted answers, as they have high levels of anxiety and hyperactive behaviour towards social interactions and new situations (Del Hoyo et al., [<reflink idref="bib20" id="ref59">20</reflink>]; Medina-Gómez & García, [<reflink idref="bib31" id="ref60">31</reflink>]; Wheeler et al., [<reflink idref="bib43" id="ref61">43</reflink>]).</p> <p>In this study, differences have been detected solely in four domains and in the total sum (the hypothesis cannot be confirmed) adults with fragile X syndrome obtained higher scores with a medium effect size in stereotyped behaviour & hyperactivity and in social isolation. Although, slightly low but significant differences were found between both groups' total average sums in maladaptive behaviour. As with other studies, people with fragile X syndrome present more stereotyped and hyperactive behaviour, and social isolation than people with Down syndrome, since, along with anxiety, these may be considered core symptoms of this syndrome (Calvo et al., [<reflink idref="bib10" id="ref62">10</reflink>]). People with fragile X syndrome suffer from anxiety mainly in situations of social interaction and it is related to the presence of maladaptive behaviours (Cordeiro et al., [<reflink idref="bib15" id="ref63">15</reflink>]). In order to reduce the level of anxiety, some of these people need to perform stereotyped behaviours, move away from the group or show motor restlessness. Besides, these behaviours are likely to persist from childhood to adulthood (Hustyi et al., [<reflink idref="bib29" id="ref64">29</reflink>]). The present study also finds that people with Down syndrome score significantly higher than people with fragile X syndrome in domains such as trustworthiness and interpersonal disrupting behaviour. In this case, though, differences are low. J. A. Artigas ([<reflink idref="bib3" id="ref65">3</reflink>]), in a purely descriptive study, observed that the most common behaviours among people with Down syndrome are challenging, hyperactive and aggressive behaviours.</p> <p>However, perhaps the most relevant information that has been obtained thanks to this study, and hence the need to take maladaptive results with caution, is the little impact of maladaptive behaviours on participants, as some do not score in these domains, probably because one of the characteristics that define maladaptive behaviour is that not all people with intellectual disability present these behaviours (Bartholomay et al., [<reflink idref="bib5" id="ref66">5</reflink>]). This could also explain the fact that reliability scores were not as high as in the first part of the scale. In fact, previous studies found that children with fragile X syndrome and Down syndrome generally show themselves more pacific and display fewer behavioural issues than children with intellectual disability of unknown aetiology, contrary to the high incidence reported in other research projects. Most of these studies use clinical criteria to make the diagnosis and consider maladaptive behaviour as a symptom of psychiatric problems (Cordeiro et al., [<reflink idref="bib16" id="ref67">16</reflink>]). As a consequence, several studies have pointed out that a great number of individuals with fragile X syndrome are treated with psychiatric medication (Blacher & McIntre, [<reflink idref="bib6" id="ref68">6</reflink>]).</p> <p>Probably, when the evaluation test is applied to analyse a person´s limitations and is managed by experts devoted to the care and support of people with intellectual disability, highly experienced in determining the phenotypical characteristics of these syndromes, maladaptive behaviour could be considered not as a psychiatric disorder but as a lack of skills. This interpretation would indicate how useful it is to employ behavioural interventions for learning skills instead of using treating maladaptive behaviour with drugs. Studies carried out in people with autism show that behavioural intervention improves intellectual and adaptive functioning and reduces the occurrence of maladaptive behaviour (Sallows & Graupner, [<reflink idref="bib38" id="ref69">38</reflink>]). Similar interventions could be a beneficial treatment option also in fragile X syndrome and in Down syndrome. The findings of this study may have major implications, as they indicate that treatment must be a primary concern for future research in the matter.</p> <p>Finally, concerning the second aim of this research, as well as Côté et al. ([<reflink idref="bib18" id="ref70">18</reflink>]), this study does not show any clear evidence of a relation between adaptive and maladaptive behaviour in fragile X syndrome, as no correlation was observed. Therefore, it could be said that either the presence or the absence of maladaptive behaviour does not affect the acquisition of conceptual, social, and practical skills. However, there is a moderate correlation (<emph>r</emph> = −.425) in people with Down syndrome and, after performing linear regression, it has been observed that 18% of adaptive behaviour is explained by maladaptive behaviour, this is because higher scores reflect more adaptive behaviour and more maladaptive behaviour. Some authors found a correlation between both behaviours in previous studies. Thus, further research is required in order to manage the influence of other factors, such as the level of IQ and environmental factors, on adaptive and maladaptive behaviour, and to clearly determine the potential influence. Also, further longitudinal studies could be useful to analyse the evolution of adaptive behaviour throughout life individual study will contribute to assess the limitations of each individual to later establish the most appropriate support interventions with the aim of improving the functioning of people with difficulties in specific skills. Findings suggest that efforts must be devoted to the prevention of maladaptive behaviours at early ages, because children with these syndromes are at a higher risk of developing behaviours deemed inappropriate.</p> <p>The results obtained could be considered an approach to the situation of people with fragile X syndrome o Down syndrome, hence the need for further research. As with every empirical study, this one has some limitations too. Previous knowledge about the phenotype of the participants could have influenced the examiners' answers to the items. Also, the scale used does not collect all the defining characteristics of the behavioural phenotype of both syndromes and some significant aspects may not have been contrasted. Moreover, it would be adviceable to use more current and validated scales in adults with disabilities.</p> <p>Even when an attempt has been made to control the influence of certain variables, such as IQ level and co-occurring disorders, it would be interesting to gain access to assessment tools which are more adapted and adequate to, and more validated in the spanish population, thus helping control those variables, as, for the moment, such tools are scarce. Despite the limitations, the results of this study are quite promising, as these findings could guide the setting of the goals of early-age intervention to increase adaptive strengths and reduce maladaptive behaviour through the design of more effective action plans and resources more adapted to each person. It is imperative to gain access to instruments which improve the care of individuals with different genetic disorders. Advances in this knowledge field should encourage further research on the influence of genetic disorders on the behaviour of people with syndromes associated with intellectual disability. As well, as the influence of other possible variables that may be affecting the results (previous experiences, opportunities, educational style, etc.).</p> <hd id="AN0187437675-9">Disclosure Statement</hd> <p>No potential conflict of interest was reported by the author(s).</p> <ref id="AN0187437675-10"> <title> References </title> <blist> <bibl id="bib1" idref="ref14" type="bt">1</bibl> <bibtext> Artigas, J., & Brun, C. (2004). ¿Se puede atribuir el fenotipo conductual del síndrome X frágil al retraso mental y al trastorno por déficit de atención/hiperactividad? Revista Neurología, 38 (1), 7 – 11. https://doi.org/10.33588/rn.3801.2003403</bibtext> </blist> <blist> <bibl id="bib2" idref="ref6" type="bt">2</bibl> <bibtext> Artigas, J. A. (2011a). Síndrome X frágil. In J. A. Artigas & J. Narbona (Eds.), Trastornos del neurodesarrollo (pp. 101 – 120). Viguera Editores.</bibtext> </blist> <blist> <bibl id="bib3" idref="ref28" type="bt">3</bibl> <bibtext> Artigas, J. A. (2011b). Otros síndromes con retraso mental. In J. A. Artigas & J. Narbona (Eds.), Trastornos del neurodesarrollo (pp. 151 – 169). Viguera Editores.</bibtext> </blist> <blist> <bibl id="bib4" idref="ref40" type="bt">4</bibl> <bibtext> Bargagna, S., Canepa, G., & Tinelli, F. (2002). Social adjustment in children with down mental retardation (MRD) and fragile-X mental retardation (MRX). Panminerva Médica, 44 (1), 7 – 10. PMID: 11887084.</bibtext> </blist> <blist> <bibl id="bib5" idref="ref7" type="bt">5</bibl> <bibtext> Bartholomay, K. L., Lee, C. H., Bruno, J. L., Lightbody, A. A., & Reiss, A. L. (2019). Closing the gender gap in fragile X syndrome: Review on females with fragile X syndrome and preliminary research findings. Brain Sciences, 9 (1), 11 – 171. https://doi.org/10.3390/brainsci9010011</bibtext> </blist> <blist> <bibl id="bib6" idref="ref68" type="bt">6</bibl> <bibtext> Blacher, J., & McIntre, L. L. (2006). Syndrome especificity and behavioural disorders in youngadults with intellectual disability: Cultural differences in family impact. Journal of Intellectual Disability Research, 50 (3), 184 – 198. https://doi.org/10.1111/j.1365-2788.2005.00768.x</bibtext> </blist> <blist> <bibl id="bib7" idref="ref11" type="bt">7</bibl> <bibtext> Brady, N. C., Fleming, K., Bredin‐Oja, S. L., Fielding‐Gebhardt, H., & Warren, S. (2020). Language development from early childhood to adolescence in youths with fragile X syndrome. Journal Speech, Lang Hear Res, 63 (11), 3727 – 3742. https://doi.org/10.1044/2020_JSLHR-20-00198</bibtext> </blist> <blist> <bibl id="bib8" idref="ref48" type="bt">8</bibl> <bibtext> Bruininks, R. H., Hill, B. K., Weatherman, R. F., & Woodcock, R. W. (1986). Examiner's manual. ICAP: Inventory for client and agency planning. DLM Teaching Resources.</bibtext> </blist> <blist> <bibl id="bib9" idref="ref23" type="bt">9</bibl> <bibtext> Burack, J. A., Shulman, C., Katzir, E., Schaap, T., Brennan, J. M., Iarocci, G., Amir, P. N., & Amir, N. (1999). Cognitive and behavioural development of Israeli males with fragile X and Down syndrome. International Journal of Behavioral Development, 23 (2), 519 – 531. https://doi.org/10.1097/tld.0b013e3181a72016</bibtext> </blist> <blist> <bibtext> Calvo, R., Quintero, C., Pérez, L., & Sánchez, L. (2014). Aspectos médicos de los pacientes con síndrome X frágil. In E. B. Medina-Gómez, I. García & Y. de Diego (Eds.), S índrome X frágil. Manual para profesionales y familiares (pp. 39 – 61). Altaria.</bibtext> </blist> <blist> <bibtext> Carrillo, M. (2013). Inteligencia, conducta adaptativa y calidad de vida. Interacciones explicativas de la discapacidad intelectual y la intervención optimizadora [ tesis doctoral ]. Universidad Autónoma de Barcelona.</bibtext> </blist> <blist> <bibtext> Chorpita, B. F. (2018). Child & adolescent psychotherapy: Components of evidence-based treatments for youth and their parents. Cambridge University press.</bibtext> </blist> <blist> <bibtext> Cohen, I. L., Richard, N., Tordjman, S., Baumann, C., Burglen, L., Excoffier, E., Lazar, G., Mazet, P., Pinquier, C., Verloes, A., & H?ron, D. (2005). Specific genetic disorders and autism: Clinical contribution towar their identification. Journal of Autism & Developmental Disorders, 35 (1), 103 – 116. https://doi.org/10.1007/s10803-004-1038-2</bibtext> </blist> <blist> <bibtext> Cohen, J. (1988). Statistical power analysis for the behavioral science (2nd ed.). Lawrence Erlbaum Associates.</bibtext> </blist> <blist> <bibtext> Cordeiro, L., Abucayan, F., Hagerman, R., Tassone, F., & Hessl, D. (2015). Anxiety disorders in fragile X premutation carriers: Preliminary characterization of probands and non-probands. Intractable Rare Disease Rearch, 4 (3), 123 – 130. https://doi.org/10.5582/irdr.2015.01029</bibtext> </blist> <blist> <bibtext> Cordeiro, L., Ballinger, E., Hagerman, R., & Hessl, D. (2011). Clinical assessment of SDM-IV anxiety disorders in fragile X syndrome: Prevalence and characterization. Journal Neurodevelopmental Disorder, 3 (1), 57 – 67. https://doi.org/10.1007/s11689-010-9067</bibtext> </blist> <blist> <bibtext> Cornish, K., Cole, V., Longhi, E., Karmiloff-Smith, A., & Scerif, G. (2013). Mapping developmental trajectories of attention and working memory in fragile X syndrome: Developmental freeze or developmental change? Development & Psychopathology, 25 (2), 365 – 376. https://doi.org/10.1017/S0954579412001113</bibtext> </blist> <blist> <bibtext> Côté, V., Knoth, I. S., Lalancette, È., Lavergne, J. A., Côté, L., Major, P., & Lippé, S. (2020). Behavioural characteristics related to adaptive functioning in young persons with tuberous sclerosis complex, down syndrome and fragile x syndrome. Journal of Developmental and Physical Disabilities, 33 (2), 279 – 296. https://doi.org/10.1007/s10882-020-09748-8</bibtext> </blist> <blist> <bibtext> De Graaf, G., Buckley, F., & Skotko, B. G. (2021). Estimation of the number of people with down syndrome in Europe. European Journal of Human Genetics, 29 (3), 402 – 410. https://doi.org/10.1038/s41431-020-00748-y</bibtext> </blist> <blist> <bibtext> Del Hoyo, L., Thurman, A. J., & Abbeduto, L. (2018). Specificity: A phenotypic comparison of communication-relevant domains between youth with down syndrome and fragile X syndrome. Frontiers in Genetics, 9, 424. https://doi.org/10.3389/fgene.2018.00424</bibtext> </blist> <blist> <bibtext> Edgin, J. O. (2013). Cognition in down syndrome: A developmental cognitive neuroscience perspective. Wiley Interdisciplinary Reviews Cognitive Science, 4 (3), 307 – 317. https://doi.org/10.1002/wcs.1221</bibtext> </blist> <blist> <bibtext> Ersoy, S. A., Güler, H. A., & Çetin, F. H. (2018). Psychopathology in down syndrome. Advances in Research on Down Syndrome, 1, 17. https://doi.org/10.5772/intechopen.71061</bibtext> </blist> <blist> <bibtext> Esbensen, A. J., Bishop, S., Seltzer, M. M., Greenberg, J. S., & Taylor, J. L. (2010). Comparisons between individuals with autisms spectrum disorders and individuals with down syndrome in adulthood. American Journal on Intellectual and Developmental, 115 (4), 277 – 290. https://doi.org/10.1352/1944-7558-115.4.277</bibtext> </blist> <blist> <bibtext> Hagerman, R., & Hagerman, P. (2013). Advances in clinical and molecular understanding of the FMR1 premutation and fragile X-associated tremor/ataxia syndrome. Lancet Neurology, 12 (8), 786 – 798. https://doi.org/10.1016/S1474-4422(13)70125-X</bibtext> </blist> <blist> <bibtext> Harries, J., Guscia, R., Kirby, N., Nettelbeck, T., & Taplin, J. (2005). Support nee SD and adaptive behaviors. Journal Information, 110 (5), 393 – 404. https://doi.org/10.1352/0895-8017(2005)110[393:SNAAB]2.0.CO;2</bibtext> </blist> <blist> <bibtext> Harrison, P. L. (1987). Research with adaptive behavior scales. Journal of Special Education, 21 (1), 37 – 68. https://doi.org/10.1352/0895-8017</bibtext> </blist> <blist> <bibtext> Hartley, S. L., Seltzer, M. M., Head, L., & Abbeduto, L. (2012). Psychological well‐being in fathers of adolescents and young adults with down syndrome, fragile X syndrome, and autism. Family Relations, 61 (2), 327 – 342. https://doi.org/10.1111/j.1741-3729.2011.00693.x</bibtext> </blist> <blist> <bibtext> Huete, A. (2016). Demografía e inclusión social de las personas con síndrome de Down. Revista Síndrome Down, 33 (129), 38 – 50. <ulink href="http://hdl.handle.net/11181/4946">http://hdl.handle.net/11181/4946</ulink></bibtext> </blist> <blist> <bibtext> Hustyi, K. M., Hall, S. S., Jo, B., Lightbody, A. A., Allan, L., & Reiss, A. L. (2014). Longitudinal trajectories of aberrant behavior in fragile X syndrome. Research in Developmental Disabilities, 35 (11), 2691 – 2701. https://doi.org/10.1016/j.ridd.2014.07.003</bibtext> </blist> <blist> <bibtext> Medina-Gómez, B. (2014). El síndrome X frágil: identificación del fenotipo y propuestas educativas. Revista Española de Discapacidad, 2 (2), 45 – 62. https://doi.org/10.5569/2340-5104.02.02.03</bibtext> </blist> <blist> <bibtext> Medina-Gómez, B., & García, I. (2016). Síndrome X frágil: detección e intervención en el fenotipo conductual. International Journal of Developmental and Educational Psychology Revista INFAD de Psicología, 2 (1), 145 – 154. https://doi.org/10.17060/ijodaep.2014.n1.v2.427</bibtext> </blist> <blist> <bibtext> Medina-Gómez, B., García, I., & Antolin, V. (2015). Validación preliminar de la Escala de Conducta Adaptativa ABS-RC: 2 en España. Acta De Investigación Psicológica, 5 (2), 2076 – 2086. https://doi.org/10.1016/s2007-4719(15)30024-7</bibtext> </blist> <blist> <bibtext> Montero, D. (1993). Evaluación de la Conducta Adaptativa en Personas con Discapacidades. Ediciones Mensajero.</bibtext> </blist> <blist> <bibtext> Moskowitiz, L. J., & Jones, E. A. (2015). Uncovering the evidence for behavioral interventions with individuals with fragile X syndrome: A systematic. Review Research in Developmental Disabilities, 38, 223 – 241. https://doi.org/10.1016/j.ridd.2014.12.011</bibtext> </blist> <blist> <bibtext> Nihira, K., Leland, H., & Lambert, N. (1993). Adaptive behaviour scale-residential and community: Second edition. Pro-Ed.</bibtext> </blist> <blist> <bibtext> Raspa, M., Wheeler, A. C., & Riley, C. (2017). Public health literature review of fragile X syndrome. Pediatrics, 139 (Suppl Supplement_3), S153 – S171. https://doi.org/10.1542/peds.2016-1159C</bibtext> </blist> <blist> <bibtext> Reches, A. (2019). Fragile X syndrome: Introduction. Methods in Molecular Biology, 1942, 3 – 10. https://doi.org/10.1007/978-1-4939-9080-1</bibtext> </blist> <blist> <bibtext> Sallows, G. O., & Graupner, T. D. (2005). Intensive behavioral treatment for children with autism: Four-year outcome and predictors. American Journal of Mental Retardation, 110 (6), 417 – 438. https://doi.org/10.1352/0895-8017(2005)110[417:IBTFCW]2.0.CO;2</bibtext> </blist> <blist> <bibtext> Schalock, R. L., Borthwick-Duffy, S. A., Bradley, V. J., Buntinx, W. H., Coulter, D. L., Craig, E. M., Gomez, S. C., Lachapelle, Y., Luckasson, R., Reeve, A., Shogren, K. A., Snell, M. E., Spreat, S., Tasse, M. J., Thompson, J. R., Verdugo-Alonso, M. A., Wehmeyer, M. L., & Yeager, M. H. (2010). Intellectual disability: Definition, classification, and systems of supports. American Association on Intellectual and Developmental Disabilities.</bibtext> </blist> <blist> <bibtext> Schalock, R. L., Luckasson, R., & Tassé, M. J. (2021). Intellectual disability: Definition, diagnosis, classification, and systems of supports (12th ed.). American Association on Intellectual and Developmental Disabilities.</bibtext> </blist> <blist> <bibtext> Smith, L. E., Erin, T., Barker, E. T., Seltzer, M. M., Abbeduto, L., & Greenberg, J. S. (2012). Behavioral phenotype of fragile X syndrome in adolescence and adulthood. The American, 117 (1), 1 – 17. https://doi.org/10.1352/1944-7558-117.1.1</bibtext> </blist> <blist> <bibtext> Wassertein, R. L., & Lazar, N. A. (2016). The ASA statement on p -values: Context, process, and purpose. American Statistician, 70 (2), 129 – 133. https://doi.org/10.1080/00031305.2016.1154108</bibtext> </blist> <blist> <bibtext> Wheeler, A., Raspa, M., Bann, C., Bishop, E., Hessl, D., Sacco, P., & Bailey, D. B., Jr. (2014). Anxiety, attention problems, hyperactivity, and the aberrant behavior checklist in fragile X syndrome. American Journal of Medical Genetics Part A, 164 (1), 141 – 155. https://doi.org/10.1002/ajmg.a.36232</bibtext> </blist> <blist> <bibtext> Zhu, Z., Li, W., Zhan, J., Hu, L., Wu, L., & Zhao, Z. (2016). Adaptive behaviour of chinese boys with fragile X syndrome. Journal of Intellectual Disability Research, 60 (1), 1 – 8. https://doi.org/10.1111/jir.12222</bibtext> </blist> </ref> <aug> <p>By Begoña Medina-Gómez and Gloria Pérez de Albéniz-Garrote</p> <p>Reported by Author; Author</p> </aug> <nolink nlid="nl1" bibid="bib40" firstref="ref1"></nolink> <nolink nlid="nl2" bibid="bib34" firstref="ref2"></nolink> <nolink nlid="nl3" bibid="bib37" firstref="ref3"></nolink> <nolink nlid="nl4" bibid="bib19" firstref="ref4"></nolink> <nolink nlid="nl5" bibid="bib24" firstref="ref5"></nolink> <nolink nlid="nl6" bibid="bib15" firstref="ref8"></nolink> <nolink nlid="nl7" bibid="bib39" firstref="ref9"></nolink> <nolink nlid="nl8" bibid="bib13" firstref="ref10"></nolink> <nolink nlid="nl9" bibid="bib36" firstref="ref12"></nolink> <nolink nlid="nl10" bibid="bib17" firstref="ref13"></nolink> <nolink nlid="nl11" bibid="bib28" firstref="ref15"></nolink> <nolink nlid="nl12" bibid="bib12" firstref="ref19"></nolink> <nolink nlid="nl13" bibid="bib30" firstref="ref21"></nolink> <nolink nlid="nl14" bibid="bib44" firstref="ref22"></nolink> <nolink nlid="nl15" bibid="bib29" firstref="ref24"></nolink> <nolink nlid="nl16" bibid="bib41" firstref="ref25"></nolink> <nolink nlid="nl17" bibid="bib43" firstref="ref26"></nolink> <nolink nlid="nl18" bibid="bib23" firstref="ref27"></nolink> <nolink nlid="nl19" bibid="bib22" firstref="ref29"></nolink> <nolink nlid="nl20" bibid="bib16" firstref="ref30"></nolink> <nolink nlid="nl21" bibid="bib32" firstref="ref31"></nolink> <nolink nlid="nl22" bibid="bib18" firstref="ref33"></nolink> <nolink nlid="nl23" bibid="bib26" firstref="ref34"></nolink> <nolink nlid="nl24" bibid="bib27" firstref="ref35"></nolink> <nolink nlid="nl25" bibid="bib21" firstref="ref39"></nolink> <nolink nlid="nl26" bibid="bib11" firstref="ref43"></nolink> <nolink nlid="nl27" bibid="bib35" firstref="ref45"></nolink> <nolink nlid="nl28" bibid="bib33" firstref="ref49"></nolink> <nolink nlid="nl29" bibid="bib42" firstref="ref50"></nolink> <nolink nlid="nl30" bibid="bib14" firstref="ref51"></nolink> <nolink nlid="nl31" bibid="bib25" firstref="ref56"></nolink> <nolink nlid="nl32" bibid="bib10" firstref="ref57"></nolink> <nolink nlid="nl33" bibid="bib20" firstref="ref59"></nolink> <nolink nlid="nl34" bibid="bib31" firstref="ref60"></nolink> <nolink nlid="nl35" bibid="bib38" firstref="ref69"></nolink>
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  Label: Title
  Group: Ti
  Data: Adaptive and Maladaptive Behaviour in Adults with Fragile X Syndrome and Down Syndrome
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  Label: Language
  Group: Lang
  Data: English
– Name: Author
  Label: Authors
  Group: Au
  Data: <searchLink fieldCode="AR" term="%22Begoña+Medina-Gómez%22">Begoña Medina-Gómez</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0002-4772-7798">0000-0002-4772-7798</externalLink>)<br /><searchLink fieldCode="AR" term="%22Gloria+Pérez+de+Albéniz-Garrote%22">Gloria Pérez de Albéniz-Garrote</searchLink> (ORCID <externalLink term="https://orcid.org/0000-0002-7776-6640">0000-0002-7776-6640</externalLink>)
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  Data: <searchLink fieldCode="SO" term="%22International+Journal+of+Disability%2C+Development+and+Education%22"><i>International Journal of Disability, Development and Education</i></searchLink>. 2025 72(6):1121-1133.
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  Data: Routledge. Available from: Taylor & Francis, Ltd. 530 Walnut Street Suite 850, Philadelphia, PA 19106. Tel: 800-354-1420; Tel: 215-625-8900; Fax: 215-207-0050; Web site: http://www.tandf.co.uk/journals
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  Label: Peer Reviewed
  Group: SrcInfo
  Data: Y
– Name: Pages
  Label: Page Count
  Group: Src
  Data: 13
– Name: DatePubCY
  Label: Publication Date
  Group: Date
  Data: 2025
– Name: TypeDocument
  Label: Document Type
  Group: TypDoc
  Data: Journal Articles<br />Reports - Research
– Name: Subject
  Label: Descriptors
  Group: Su
  Data: <searchLink fieldCode="DE" term="%22Behavior+Problems%22">Behavior Problems</searchLink><br /><searchLink fieldCode="DE" term="%22Adults%22">Adults</searchLink><br /><searchLink fieldCode="DE" term="%22Down+Syndrome%22">Down Syndrome</searchLink><br /><searchLink fieldCode="DE" term="%22Genetic+Disorders%22">Genetic Disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Foreign+Countries%22">Foreign Countries</searchLink><br /><searchLink fieldCode="DE" term="%22Daily+Living+Skills%22">Daily Living Skills</searchLink><br /><searchLink fieldCode="DE" term="%22Interpersonal+Relationship%22">Interpersonal Relationship</searchLink><br /><searchLink fieldCode="DE" term="%22Social+Behavior%22">Social Behavior</searchLink><br /><searchLink fieldCode="DE" term="%22Antisocial+Behavior%22">Antisocial Behavior</searchLink><br /><searchLink fieldCode="DE" term="%22Correlation%22">Correlation</searchLink>
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  Label: Geographic Terms
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  Data: <searchLink fieldCode="DE" term="%22Spain%22">Spain</searchLink>
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  Label: Assessment and Survey Identifiers
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  Data: <searchLink fieldCode="SU" term="%22Adaptive+Behavior+Scale%22">Adaptive Behavior Scale</searchLink>
– Name: DOI
  Label: DOI
  Group: ID
  Data: 10.1080/1034912X.2024.2427616
– Name: ISSN
  Label: ISSN
  Group: ISSN
  Data: 1034-912X<br />1465-346X
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: This study assesses adaptive and maladaptive behaviour in a sample of 68 adults. The Spanish version of the Adaptive Behaviour Scale: Residence and Community (ABS-RC:2) was applied to 30 subjects with Fragile X Syndrome (FXS) and 38 subjects with Down Syndrome (DS) aged between 25 and 57. The results show that adults with FXS score higher than do those with DS in some adaptive behaviours, such as physical development and economic activities while scoring less than adults with DS in self-direction. Adults with FXS show more stereotyped behaviours, hyperactivity, and social isolation, but less trustworthiness and disturbing interpersonal behaviours than do those with DS. A relation was found between both behaviours in DS: the higher the scores in maladaptive behaviour, the lower those in adaptive behaviour. However, no such relation was found in individuals with FXS.
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  Label: Entry Date
  Group: Date
  Data: 2025
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  Label: Accession Number
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  Data: EJ1490777
PLink https://search.ebscohost.com/login.aspx?direct=true&site=eds-live&db=eric&AN=EJ1490777
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  BibEntity:
    Identifiers:
      – Type: doi
        Value: 10.1080/1034912X.2024.2427616
    Languages:
      – Text: English
    PhysicalDescription:
      Pagination:
        PageCount: 13
        StartPage: 1121
    Subjects:
      – SubjectFull: Behavior Problems
        Type: general
      – SubjectFull: Adults
        Type: general
      – SubjectFull: Down Syndrome
        Type: general
      – SubjectFull: Genetic Disorders
        Type: general
      – SubjectFull: Foreign Countries
        Type: general
      – SubjectFull: Daily Living Skills
        Type: general
      – SubjectFull: Interpersonal Relationship
        Type: general
      – SubjectFull: Social Behavior
        Type: general
      – SubjectFull: Antisocial Behavior
        Type: general
      – SubjectFull: Correlation
        Type: general
      – SubjectFull: Spain
        Type: general
      – SubjectFull: Adaptive Behavior Scale
        Type: general
    Titles:
      – TitleFull: Adaptive and Maladaptive Behaviour in Adults with Fragile X Syndrome and Down Syndrome
        Type: main
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          Name:
            NameFull: Begoña Medina-Gómez
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          Name:
            NameFull: Gloria Pérez de Albéniz-Garrote
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            – D: 01
              M: 01
              Type: published
              Y: 2025
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            – Type: issn-print
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              Value: 1465-346X
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              Value: 72
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              Value: 6
          Titles:
            – TitleFull: International Journal of Disability, Development and Education
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