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ADHD Symptoms and Insistence on Sameness in Prader-Willi Syndrome

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Title: ADHD Symptoms and Insistence on Sameness in Prader-Willi Syndrome
Language: English
Authors: Wigren, M., Hansen, S.
Source: Journal of Intellectual Disability Research. Jun 2005 49(6):449-456.
Availability: Journal Customer Services, Blackwell Publishing, 350 Main Street, Malden, MA 02148. Tel: 800-835-6770 (Toll Free); Fax: 781-388-8232; e-mail: subscrip@bos.blackwellpublishing.com.
Peer Reviewed: Y
Page Count: 8
Publication Date: 2005
Document Type: Journal Articles
Reports - Research
Education Level: Elementary Secondary Education
Descriptors: Profiles, Eating Disorders, Symptoms (Individual Disorders), Attention Deficit Disorders, Hyperactivity, Disabilities, Antisocial Behavior, Children, Adolescents, Rating Scales
DOI: 10.1111/j.1365-2788.2005.00690.x
ISSN: 0964-2633
Abstract: Background: Apart from a pervasive eating disorder, the Prader-Willi (PWS) syndrome is characterized by a distinct behavioural profile comprising maladaptive behaviours, obsessive-compulsive traits and skin picking, all included in the PWS behavioural phenotype. In this study, we present a further delineation of this characteristic behavioural profile by screening for indices of executive dysfunctions related to attention-deficit hyperactivity disorder (ADHD), immature compulsive-like adherence to sameness and skin picking, and how these features aggregate into symptom constellations in children and adolescents with PWS. Method: Parents of 58 individuals with PWS (aged 5-18 years) participated by completing Childhood Routines Inventory (CRI) and Conners Parent Rating Scale (CPRS-48). Results: Results showed that indices of ADHD and excessive insistence on sameness were common, comorbid and of early onset. They were both associated with conduct problems. Skin picking, appearing as a single and comorbid symptom, was less associated with childlike compulsions and ADHD-related problems. Conclusions: Findings are discussed in terms of further research in executive dysfunctions in PWS.
Abstractor: Author
Number of References: 39
Entry Date: 2005
Accession Number: EJ718421
Database: ERIC
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  Value: <anid>AN0016975451;eul01jun.05;2019Jun04.10:26;v2.2.500</anid> <title id="AN0016975451-1">ADHD symptoms and insistence on sameness in Prader-Willi syndrome. </title> <p>Background  Apart from a pervasive eating disorder, the Prader‐Willi (PWS) syndrome is characterized by a distinct behavioural profile comprising maladaptive behaviours, obsessive‐compulsive traits and skin picking, all included in the PWS behavioural phenotype. In this study, we present a further delineation of this characteristic behavioural profile by screening for indices of executive dysfunctions related to attention‐deficit/hyperactivity disorder (ADHD), immature compulsive‐like adherence to sameness and skin picking, and how these features aggregate into symptom constellations in children and adolescents with PWS. Method  Parents of 58 individuals with PWS (aged 5–18 years) participated by completing Childhood Routines Inventory (CRI) and Conners' Parent Rating Scale (CPRS‐48). Results  Results showed that indices of ADHD and excessive insistence on sameness were common, comorbid and of early onset. They were both associated with conduct problems. Skin picking, appearing as a single and comorbid symptom, was less associated with childlike compulsions and ADHD‐related problems. Conclusions  Findings are discussed in terms of further research in executive dysfunctions in PWS.</p> <p>Keywords: Childhood Routines Inventory; CPRS‐48; executive functions; Prader‐Willi syndrome; ADHD</p> <p>The Prader‐Willi syndrome (PWS) is a rare genetically determined condition, caused by abnormalities in chromosome 15. The syndrome is characterized by incomplete or delayed pubertal development, short stature, difficulties in gross motor skills, sleep abnormalities and cognitive delays or dysfunctions. Neonatal hypotonia causes feeding problems and poor weight gain. Later on, childhood onset of hyperphagia, occurring almost universally, results in serious obesity and secondary medical complications if not controlled ([<reflink idref="bib30" id="ref1">30</reflink>]; [<reflink idref="bib3" id="ref2">3</reflink>]).</p> <p>Beside the eating disorder, the PWS condition is characterized by a distinctive behavioural profile, comprising maladaptive behaviours, obsessive‐compulsive traits and skin picking. This triad of symptoms, which constitutes a part of the PWS behavioural phenotype, has an early onset and is independent of the eating disorder, cognitive functioning and gender in PWS populations ([<reflink idref="bib12" id="ref3">12</reflink>], [<reflink idref="bib10" id="ref4">10</reflink>]; [<reflink idref="bib8" id="ref5">8</reflink>]). The underlying dysfunctions responsible for these ancillary symptoms are unknown but the early onset in combination with its neuropsychiatric overtones warrants a neurodevelopmental perspective when examining its origin, nature and course. Overall, previous research indicates that individuals with PWS are impaired with respect to flexibility, response inhibition, social understanding and cognitive set shifting, i.e. impairments in executive functions in which the frontal cortex plays a critical role ([<reflink idref="bib16" id="ref6">16</reflink>]; [<reflink idref="bib20" id="ref7">20</reflink>]).</p> <p>In the present study we delineate the symptom triad by screening for indices of executive dysfunctions in order to get a better understanding of the PWS behavioural profile. More specifically, we wanted to explore if maladaptive behaviours, as measured by the CPRS‐48 Conduct problem subscale ([<reflink idref="bib5" id="ref8">5</reflink>]), can be understood in the context of ADHD‐related problems. Secondly, we investigated whether the compulsive‐like insistence on sameness is associated with indices of ADHD and/or with skin picking. Finally, we explored the extent to which ADHD‐related problems, insistence on sameness and skin picking aggregate into symptom constellations.</p> <p>Maladaptive behaviours in the PWS behavioural phenotype comprise emotional lability, temper tantrums and violent outbursts with verbal and physical aggression. Indices of hyperactivity and impulsivity have also been reported in child and adult populations ([<reflink idref="bib11" id="ref9">11</reflink>]; [<reflink idref="bib18" id="ref10">18</reflink>]; [<reflink idref="bib3" id="ref11">3</reflink>]). These maladaptive behaviours resemble those seen in children with ADHD‐related problems. In <emph>Diagnostic and Statistical Manual</emph> (DSM‐IV), ADHD covers a broad problem area and diagnostic criteria comprise three dimensions: the predominantly inattentive, predominantly hyperactive–impulsive and the combined type ([<reflink idref="bib1" id="ref12">1</reflink>]; [<reflink idref="bib24" id="ref13">24</reflink>]). The prevalence rates of ADHD are substantially enhanced in a number of neuropsychiatric conditions ([<reflink idref="bib23" id="ref14">23</reflink>]; [<reflink idref="bib38" id="ref15">38</reflink>]; [<reflink idref="bib29" id="ref16">29</reflink>]), but rigorous research on the prevalence of ADHD in PWS is sparse (but see [<reflink idref="bib18" id="ref17">18</reflink>]; [<reflink idref="bib32" id="ref18">32</reflink>]).</p> <p>In many ways the obsessive‐compulsive traits in the PWS are distinct from diagnostic criteria defining obsessive‐compulsive disorder (OCD; [<reflink idref="bib1" id="ref19">1</reflink>]), in that washing compulsions and concerns about dirt/cleanliness are rare in PWS. Instead, the compulsions are similar to the rather benign and transient rituals regularly seen in normative child development ([<reflink idref="bib4" id="ref20">4</reflink>]). Recent findings reveal that individuals with PWS were more insistent on sameness in daily routines than were healthy 4‐year‐olds. In this regard they not only fail to show the typical decline over age seen in normal childhood, but also score appreciably higher than healthy children ([<reflink idref="bib35" id="ref21">35</reflink>]). Interestingly, [<reflink idref="bib16" id="ref22">16</reflink>]) showed that in normal children, high levels of compulsive rituals are associated with impaired executive functioning, like poor response inhibition and difficulties in changing cognitive sets. This finding suggests that ADHD‐like executive deficits might be linked to compulsivity in PWS. A second important aim of the present study therefore was to explore the relationship between insistence on sameness on the one hand, and indices of ADHD and skin picking on the other.</p> <p>Skin picking is a predominant symptom in the PWS behavioural phenotype. It has an early onset, shows stability over time and prevalence rates range from 65% to 95% ([<reflink idref="bib17" id="ref23">17</reflink>]; [<reflink idref="bib31" id="ref24">31</reflink>]; [<reflink idref="bib37" id="ref25">37</reflink>]). It is presently unclear whether skin picking in PWS is best regarded as part of an early compulsive clinical picture ([<reflink idref="bib13" id="ref26">13</reflink>]), or it forms an independent symptom dimension associated with mood swings ([<reflink idref="bib17" id="ref27">17</reflink>]; [<reflink idref="bib19" id="ref28">19</reflink>]). In this study we were interested to know how and to what extent skin picking relates to the compulsive features and to the ADHD‐like symptoms.</p> <hd id="AN0016975451-2">Materials and methods</hd> <p></p> <hd id="AN0016975451-3">Participants</hd> <p>A nationwide cohort of 58 individuals with PWS, 31 boys and 27 girls, was included in this study. Additional three parents of children from the age cohort refused participating. In total, the sample group constitutes about 80% of the estimated total Swedish group of affected 5‐ to 18‐year‐olds (see [<reflink idref="bib33" id="ref29">33</reflink>]). Mean age for boys was 12.61 years (SD = 4.06; range = 5–18 years) and for girls 10.59 years (SD = 4.17; range = 5–18 years). All had confirmed PWS diagnoses via methylation analyses. A total of 71% (<emph>n</emph> = 41) were on current growth hormone treatment and additional three individuals had finished this treatment. All participants lived with their families and were referred to local habilitation departments on the basis of their PWS diagnoses. All were receiving medical and paramedical services and special educational support in local school and preschool settings.</p> <hd id="AN0016975451-4">Measures</hd> <p>Conners' Parent Rating Scale‐48 (CPRS‐48; Conners, 1989) was used to measure ADHD‐related problems and maladaptive behaviours. The CPRS is a parent‐completed 48‐item questionnaire, normed for each gender from 3 to 17 years. It comprises five subscales capturing anxiety, conduct, impulsive–hyperactive and learning problems, and psychosomatic symptoms. The CPRS‐48 also includes a 10‐item Hyperactivity Index (hereinafter denoted as ADHD Index), widely used for screening for ADHD in neuropsychiatric research ([<reflink idref="bib21" id="ref30">21</reflink>]; [<reflink idref="bib6" id="ref31">6</reflink>]; [<reflink idref="bib22" id="ref32">22</reflink>]). The Impulsive–Hyperactive subscale, which includes items from the ADHD Index, separates the impulsive–hyperactive dimension from the wider ADHD problem area. The CPRS‐48 gives standardized T‐scores. Scores above 70 are 'clinically significant', whereas those ranging from 66 to 70 are classified as 'clinically elevated'. Individuals with T‐scores ranging from 66 to 70 were included in the categorical analysis in the present study.</p> <p>The Childhood Routines Inventory (CRI; [<reflink idref="bib15" id="ref33">15</reflink>]), version 1.2 was used to assess childhood‐like compulsivity. The CRI is a parent‐directed 19‐item questionnaire used in recent research exploring compulsive‐like behaviours in normal childhood ([<reflink idref="bib15" id="ref34">15</reflink>]; [<reflink idref="bib39" id="ref35">39</reflink>]; [<reflink idref="bib27" id="ref36">27</reflink>]). [<reflink idref="bib35" id="ref37">35</reflink>]) showed that individuals with PWS score high on a specific constellation of CRI items (the Insistence on sameness subscale, including the items 1, 5, 7, 8, 10 and 17), all measuring childlike repetitive behaviours related to insistence on sameness in various daily activities. In the categorical analysis normative scores for 200 healthy 4‐ to 6‐year‐olds on the Insistence on sameness subscale (mean = 2.70 SD = 0.60; [<reflink idref="bib34" id="ref38">34</reflink>]) were used to set putative cut‐off scores for excessive rates of insistence on sameness in PWS.</p> <p>Prevalence of skin picking was measured by the item 'inflicting sores by picking and scratching the skin' on a 1–5 point scale (never, rarely, sometimes, often, always) taken from the clinical and diagnostic questionnaire. In the categorical analysis, those subjects with responses ≥3 (sometimes, often, always) were judged to exhibit problematic levels of skin picking.</p> <hd id="AN0016975451-5">Procedure</hd> <p>Four questionnaires were mailed to PWS parents: the CPRS‐48, the CRI and two additional questionnaires focusing on diagnostic issues and family service needs. Data from the latter inventories are presented elsewhere ([<reflink idref="bib36" id="ref39">36</reflink>]). Target parents were recruited from all Swedish habilitation departments and the National Prader‐Willi Syndrome Association under the Swedish National Association for Disabled Children and Young People (RBU). Twenty‐two parents participated in a test–retest procedure (<emph>n</emph> = 26; response rate = 85%; time interval = 2–4 weeks). Reliability for parents' ratings on CPRS‐48 total scores was 0.93 and 0.83–0.91 for included subscales. Corresponding figures for the CRI were 0.93 for total scale and 0.83 for the Insistence on sameness subscale (see also [<reflink idref="bib34" id="ref40">34</reflink>]).</p> <hd id="AN0016975451-6">Results</hd> <p></p> <hd id="AN0016975451-7">Maladaptive behaviours, indices of impulsivity/hyperactivity and ADHD‐related problems</hd> <p>Table 1 shows the distribution of CPRS scores as a function of severity across subscales. It can be seen that 26% reached clinically significant scores and additional 12% displayed clinically elevated scores on the ADHD Index. Findings from the Impulsive–Hyperactive subscale disclose that 19% had significant clinical scores on this subscale. Results also revealed that all individuals with high scores on the Impulsive–Hyperactive subscale had clinical significant scores on the broader ADHD Index. Worth mentioning is that four children, aged 5–13 years, had scores below or much below average on the impulsive–hyperactive dimension.</p> <p>1 Distribution of  T‐scores across CPRS subscales for the PWS group</p> <p> <ephtml> <table><thead valign="bottom"><tr><th><bold>CPRS subscales</bold></th><th><bold>Clinically significant T‐scores >70 <italic>n</italic> (%)</bold></th><th><bold>Clinically elevated T‐scores 66–70 <italic> n</italic> (%)</bold></th><th><bold>Above average T‐scores 65–61 <italic>n</italic> (%)</bold></th><th><bold>Average T‐scores 60–40 <italic>n</italic> (%)</bold></th><th><bold>Below average T‐scores <40 <italic>n</italic> (%)</bold></th></tr></thead><tbody valign="top"><tr><td>Conduct Problem</td><td>13 (22.4)</td><td>9 (15.5)</td><td> 6 (10.3)</td><td>30 (51.7)</td><td>–</td></tr><tr><td>Impulsive–Hyperactive</td><td>11 (19.0)</td><td>2 (3.4)</td><td> 6 (10.3)</td><td>35 (60.3)</td><td>4 (6.9)</td></tr><tr><td>ADHD Index</td><td>15 (25.9)</td><td>7 (12.1)</td><td>13 (22.4)</td><td>23 (39.7)</td><td>–</td></tr><tr><td>Anxiety</td><td> 2 (3.4)</td><td>1 (1.7)</td><td>–</td><td>54 (93.1)</td><td>1 (1.7)</td></tr></tbody></table> </ephtml> </p> <p>In this study, 38% had clinically elevated or significant scores on the Conduct Problem subscale. Maladaptive conduct problems often co‐occurred with ADHD‐related problems. In fact, 73% of those with clinically significant scores on the ADHD Index had excessive conduct problems. Equally interesting, Table 1 also shows that 60% had no impulsive–hyperactive problems, more than 50% scored within the normal range on the Conduct Problem subscale and about 40% had average scores on the ADHD Index. Signs of anxiety were rare in that about 95% had normal anxiety scores. In fact 26% of the total group had average or below average scores on all included CPRS‐48 subscales.</p> <hd id="AN0016975451-8">Insistence on sameness and skin picking</hd> <p>Table 2 shows the distribution of mean scores on the Insistence on sameness subscale for the PWS group and, for comparative purposes, the score distribution for healthy preschool children (see Materials and Methods). Mean scores were significantly higher for the PWS group (means = 3.77 and 2.70; <emph>t</emph> = 9.755, df = 77.668, <emph>P</emph> = 0.0004; equal variances not assumed). As seen in Table 2, 22% of the PWS group fall within the normal range for healthy children (mean ± 1 SD) and additional two individuals scored below average. In contrast, 48% (<emph>n</emph> = 28) scored extremely high (3 and 4 SDs > mean). anova revealed that raw scores for the PWS group on the Insistence on sameness subscale did not differ when partitioned across age (<emph>F</emph><subs>2,55</subs> = 9.14; <emph>P</emph> = 0.407).</p> <p>2 Distribution of mean scores for healthy children and the PWS group on the Insistence on sameness subscale</p> <p> <ephtml> <table><thead valign="bottom"><tr><th /><th><bold>4 SD > Mean >4.50 <italic>n</italic> (%)</bold></th><th><bold>3 SD > Mean 4.50–3.91 <italic>n</italic> (%)</bold></th><th><bold>2 SD > Mean 3.90–3.31 <italic>n</italic> (%)</bold></th><th><bold>Mean ± 1 SD 3.30–2.10 <italic>n</italic> (%)</bold></th><th><bold>2 SD < Mean 2.09–1.60 <italic>n</italic> (%)</bold></th><th><bold>3 SD < Mean <1.60 <italic>n</italic> (%)</bold></th></tr></thead><tbody valign="top"><tr><td>Healthy group</td><td>–</td><td> 3 (1.5)</td><td>32 (16.0)</td><td>130 (65.0)</td><td>31 (15.5)</td><td>4 (2.0)</td></tr><tr><td>PWS group</td><td>8 (13.8)</td><td>20 (34.5)</td><td>15 (25.9)</td><td> 13 (22.4)</td><td> 1 (1.7)</td><td>1 (1.7)</td></tr></tbody></table> </ephtml> </p> <p>Forty‐five individuals (78%) displayed skin picking albeit at various intensity levels. The symptom was seldom present in four cases and 12 individuals displayed this behaviour 'sometimes'. Frequent and constant skin picking was exhibited by one‐half of the total group. Given the stringent definition of skin picking (i.e. inflicting sores) used in the present study, the finding indicates that this symptom constitutes a serious health problem.</p> <hd id="AN0016975451-9">Interrelationships between conduct problems, indices of ADHD and impulsivity–hyperactivity, i...</hd> <p></p> <hd id="AN0016975451-10">Correlational analysis</hd> <p>Scores on the Conduct Problem subscale correlated with the ADHD Index (rho = 0.75; <emph>P</emph> = 0.0004), the Impulsive–Hyperactive subscale (rho = 0.60; <emph>P</emph> = 0.0004), Insistence on sameness (rho = 0.45; <emph>P</emph> = 0.0004). Scores from the Insistence on sameness subscale were associated with the ADHD Index (rho = 0.49; <emph>P</emph> = 0.0004), but less with the Impulsive–Hyperactive subscale (rho = 0.29; <emph>P</emph> = 0.023). Finally, skin picking was not related to ADHD, nor to the Impulsive–Hyperactive subscale, but correlated moderately with scores from the Insistence on sameness (rho = 0.29, <emph>P</emph> = 0.026) and Conduct Problem subscales (rho = 0.30; <emph>P</emph> = 0.020).</p> <hd id="AN0016975451-11">Categorical analysis</hd> <p>High‐scoring individuals on indices of ADHD, Insistence on sameness, and skin picking were identified according to the criteria defined in <emph>Meterials and Methods</emph>. Table 3 shows the results of this analysis for three age groups. Overall, the analysis revealed that 14% of the PWS group had no elevated problems whatsoever. One in three had either indices of ADHD, Insistence on sameness or skin picking, the latter being predominant. More than 20% were afflicted with the full triad of symptoms. It is noteworthy that this symptom constellation was present also in the youngest age group.</p> <p>3 Symptom constellations involving indices of ADHD, insistence on sameness and skin picking across age groups</p> <p> <ephtml> <table><thead valign="bottom"><tr><th><bold>Symptom constellations</bold></th><th><bold>Age 5–8 <italic>n</italic> (%)</bold></th><th><bold>Age 9–13 <italic>n</italic> (%)</bold></th><th><bold>Age 14–18 <italic>n</italic> (%)</bold></th><th><bold>All <italic>n</italic> (%)</bold></th></tr></thead><tbody valign="top"><tr><td>No elevated symptoms</td><td> 4 (22)</td><td> 3 (16)</td><td> 1 (5)</td><td> 8 (14)</td></tr><tr><td>Sameness* only</td><td> 2 (11)</td><td> 1 (5)</td><td>–</td><td> 3 (5)</td></tr><tr><td>ADHD† only</td><td> 1 (6)</td><td> 1 (5)</td><td> 1 (5)</td><td> 3 (5)</td></tr><tr><td>Skin picking‡ only</td><td> 6 (33)</td><td> 6 (32)</td><td> 3 (14)</td><td>15 (26)</td></tr><tr><td>Sameness + ADHD</td><td> 1 (6)</td><td>–</td><td> 2 (10)</td><td> 3 (5)</td></tr><tr><td>Sameness + skin picking</td><td> 1 (6)</td><td> 4 (21)</td><td> 5 (24)</td><td>10 (17)</td></tr><tr><td>ADHD + skin picking</td><td> 1 (6)</td><td> 1 (5)</td><td> 2 (10)</td><td> 4 (7)</td></tr><tr><td>ADHD + sameness + skin picking</td><td> 2 (11)</td><td> 3 (16)</td><td> 7 (33)</td><td>12 (21)</td></tr><tr><td>Total</td><td>18 (100)</td><td>19 (100)</td><td>21 (100)</td><td>58 (100)</td></tr></tbody></table> </ephtml> </p> <p>1 * Sameness = mean scores above 3.90 on the Insistence on sameness subscale from the CRI.</p> <ulist> <item>2 † ADHD = indices of ADHD; T‐scores above 65 on the CPRS‐48 Hyperactivity Index subscale.</item> <item>3 ‡ Skin picking = 'sometimes', 'often', 'always'.</item> </ulist> <hd id="AN0016975451-12">Discussion</hd> <p>This study screened for indices of executive dysfunctions by focusing on ADHD‐related features and compulsive‐like adherence to sameness in children and adolescents with PWS. One central finding was that one‐fourth of our sample showed clinical indices of ADHD, which in some cases were present from an early age. Thus our results challenge the impression reported by [<reflink idref="bib13" id="ref41">13</reflink>]) who suggested that clinically significant rates of ADHD are not observed in people with PWS. Furthermore, findings from the present study show that signs of ADHD were highly associated with maladaptive conduct problems, indicating that these co‐occurring features form a significant psychological problem in youngsters with PWS. The finding is not surprising as manifestations of ADHD with comorbid disruptive behaviours are common in many neurospychiatric disorders ([<reflink idref="bib23" id="ref42">23</reflink>]; [<reflink idref="bib38" id="ref43">38</reflink>]; [<reflink idref="bib29" id="ref44">29</reflink>]).</p> <p>Results also show that 22% had significant problems with hyperactivity and impulsivity. At face value, this finding is at variance with previous research reporting prevalence of withdrawal, lack of energy and under‐activity in PWS. While tantrums and maladaptive behaviours decrease in adulthood, lethargy and under‐activity are phenomena that seem to increase with age ([<reflink idref="bib3" id="ref45">3</reflink>]; [<reflink idref="bib9" id="ref46">9</reflink>]). In this young population only 7% show indices of under‐activity by scoring below average on the Impulsive–Hyperactive subscale. In PWS, maladaptive behaviours might to some extent result from insatiable hunger and psychosocial consequences of being affected. Nevertheless, the observed high rates of indices of ADHD and hyperactive problems and their association with conduct problems indicate that part of the maladaptive behaviour profile is secondary to impairments in executive functions.</p> <p>A second central finding from this study concerned the high level of childlike insistence on sameness. In about 50% of the cases, regardless of chronological age, the intensity levels were similar to those observed in typically developing preschoolers. These individuals might follow the same developmental path as healthy children but at a slower rate. According to this line of reasoning, children with PWS aged 5–8 years in the present study display normal or delayed repetitive insistence on sameness, while the adolescents, being engaged in these activities to an equal, 'childlike' extent, show clear indices of arrested development (see [<reflink idref="bib19" id="ref47">19</reflink>]). Similar delayed developmental course of childlike compulsive patterns is also observed in individuals with Down syndrome ([<reflink idref="bib14" id="ref48">14</reflink>]).</p> <p>In the present study, most of the remaining individuals exhibited exceedingly high levels of insistence on sameness, rarely seen in normal development. These high rates might also be related to an arrested or delayed development, but in this case the compulsions are augmented by environmental factors, such as strict parental control in diet restrictions, household schedules and predictable daily routines ([<reflink idref="bib25" id="ref49">25</reflink>]). From this perspective, both family and child‐generated adherence to routines may serve important coping functions in balancing impairments stemming from ADHD‐related problems in the PWS child. An alternative view is based on the significant positive relationship between executive deficits (as measured by the ADHD Index) and the intensity of childlike compulsions. This indicates that pronounced executive dysfunctions might intensify the compulsive symptoms in PWS to abnormal levels. In typically developing children, unusually high levels of rituals are associated with deficient executive functioning ([<reflink idref="bib16" id="ref50">16</reflink>]).</p> <p>In previous research ([<reflink idref="bib28" id="ref51">28</reflink>]) repetitive behavioural patterns and skin picking in PWS have been associated with OCD which is a condition categorized as an anxiety disorder ([<reflink idref="bib1" id="ref52">1</reflink>]). Interestingly, our results showed that anxiety were extremely rare in this nationwide sample (see also [<reflink idref="bib7" id="ref53">7</reflink>]; [<reflink idref="bib32" id="ref54">32</reflink>]). The presence of insistence of sameness without anxiety suggests that these two entities are unrelated in this genetic condition. Furthermore, this study shows that compulsive‐like insistence on sameness was less associated with skin picking. Instead, the categorical and correlational analyses indicate that skin picking, appearing as both a single and comorbid symptom, forms a separate dimension that seem to be more associated with recurrent mood swings than to repetitive sameness patterns ([<reflink idref="bib17" id="ref55">17</reflink>]; [<reflink idref="bib19" id="ref56">19</reflink>]).</p> <p>The categorical analysis also revealed that the number of individuals with no signs of elevated problems declines with age (Table 3). The opposite age‐trend is observed at the other extreme, i.e. those clinically affected in all domains. At face value, the trends might be more apparent than real, and mostly because of the steady improvement in the clinical management of people with PWS available throughout life in the younger children but only for part of it in the older adolescents. However, although based on cross‐sectional data, an alternative and more plausible interpretation would be that these trends might reflect a time‐dependent progression inherent in the disease, such that it affects an increasingly larger number of executive functions. Even though some of the constituent clinical features superficially resemble certain disorders described in DSM‐IV ([<reflink idref="bib1" id="ref57">1</reflink>]), it would seem less advisable to consider them in traditional psychiatric terms. A more fruitful approach might be to conceptualize them as integral features in a PWS spectrum, the span of which is determined by the specific nature of the genetic condition and, not least, by the clinical management of the individual patient.</p> <p>The present study raises methodological considerations related to research on rare intellectual disability syndromes. In order to reach a large nationwide sample, cross‐sectional data were collected using postal questionnaires and parents were informants. Although ratings had solid external reliability, the indices of ADHD and insistence on sameness emerging from this study warrant further validation in clinical assessment and longitudinal research.</p> <p>Additional limitations concern the use of measures developed for populations with no intellectual disability. Firstly, the CPRS‐48 and the CRI are rough screening instruments previously not run on PWS populations. When used to portray intellectual disability syndromes, such instruments may produce factor structures different from those originally proposed. In this study, the Insistence on sameness subscale did not fully correspond to the original Repetitive Behavior subscale from the CRI, mainly because of exclusion of eating‐related items ([<reflink idref="bib15" id="ref58">15</reflink>]; [<reflink idref="bib35" id="ref59">35</reflink>]). Secondly, it is important to realize that high scores on the CPRS‐48 ADHD Index per se by no means imply confirmed diagnoses. Rather they serve as a signal for further research, clinical attention and rigorous assessment ([<reflink idref="bib6" id="ref60">6</reflink>]).</p> <p>PWS presents with two main genotypes, the paternal deletion and maternal disomy and genotypic differences are observed in that symptom intensity in maladaptive behaviours, obsessive traits and skin picking is more pronounced in individuals with paternal deletion relative to those with maternal disomy ([<reflink idref="bib2" id="ref61">2</reflink>]; [<reflink idref="bib10" id="ref62">10</reflink>]). According to Swedish clinical consensus, PWS diagnoses are assessed with methylation tests, not differentiating between genotypic subgroups ([<reflink idref="bib26" id="ref63">26</reflink>]). The present research design did not allow for genetic subtype grouping. Therefore, the executive dysfunction evidenced by intense adherence to sameness and ADHD‐related problems are of relevance for genetic subtype delineation and comparative research.</p> <p>With these cautionary notes in mind, the present study strongly suggests that ADHD‐like problems in executive control form a tangible problem in PWS. Further longitudinal research is needed in order to clarify the developmental course of 'normal' and delayed vs. deviant, excessive rates of childlike insistence on sameness in PWS. Future research is also needed to determine whether PWS is associated with predominantly the inattentive or hyperactive–impulsive type of the ADHD disorder. An important avenue would be, using neuropsychological tools, to specify the exact nature of ADHD‐related dysfunctions, such as cognitive set‐shifting and response inhibition. The way in which such executive deficits influence the propensity to show compulsive behaviour is another avenue for further research. From a clinical perspective, such studies are important because the inattentive vs. hyperactive–impulsive variants may require different intervention strategies in educational planning, in handling the eating disorder and in family guidance and support.</p> <hd id="AN0016975451-13">Acknowledgements</hd> <p>This study was supported by grants from the Swedish National Board of Health and Welfare and from the Linneá and Josef Carlsson Foundation to Margareta Wigren. The authors wish to thank parents, habilitation departments and the Swedish National Association for Disabled Children and Young People (RBU) for participating in this study.</p> <ref id="AN0016975451-14"> <title> References </title> <blist> <bibl id="bib1" idref="ref12" type="bt">1</bibl> <bibtext> American Psychiatric Association (APA) (1994) Diagnostic and Statistical Manual of Mental Disorders DSM, 4th edn. 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Hansen</p> <p>Reported by Author; Author</p> </aug> <nolink nlid="nl1" bibid="bib30" firstref="ref1"></nolink> <nolink nlid="nl2" bibid="bib12" firstref="ref3"></nolink> <nolink nlid="nl3" bibid="bib10" firstref="ref4"></nolink> <nolink nlid="nl4" bibid="bib16" firstref="ref6"></nolink> <nolink nlid="nl5" bibid="bib20" firstref="ref7"></nolink> <nolink nlid="nl6" bibid="bib11" firstref="ref9"></nolink> <nolink nlid="nl7" bibid="bib18" firstref="ref10"></nolink> <nolink nlid="nl8" bibid="bib24" firstref="ref13"></nolink> <nolink nlid="nl9" bibid="bib23" firstref="ref14"></nolink> <nolink nlid="nl10" bibid="bib38" firstref="ref15"></nolink> <nolink nlid="nl11" bibid="bib29" firstref="ref16"></nolink> <nolink nlid="nl12" bibid="bib32" firstref="ref18"></nolink> <nolink nlid="nl13" bibid="bib35" firstref="ref21"></nolink> <nolink nlid="nl14" bibid="bib17" firstref="ref23"></nolink> <nolink nlid="nl15" bibid="bib31" firstref="ref24"></nolink> <nolink nlid="nl16" bibid="bib37" firstref="ref25"></nolink> <nolink nlid="nl17" bibid="bib13" firstref="ref26"></nolink> <nolink nlid="nl18" bibid="bib19" firstref="ref28"></nolink> <nolink nlid="nl19" bibid="bib33" firstref="ref29"></nolink> <nolink nlid="nl20" bibid="bib21" firstref="ref30"></nolink> <nolink nlid="nl21" bibid="bib22" firstref="ref32"></nolink> <nolink nlid="nl22" bibid="bib15" firstref="ref33"></nolink> <nolink nlid="nl23" bibid="bib39" firstref="ref35"></nolink> <nolink nlid="nl24" bibid="bib27" firstref="ref36"></nolink> <nolink nlid="nl25" bibid="bib34" firstref="ref38"></nolink> <nolink nlid="nl26" bibid="bib36" firstref="ref39"></nolink> <nolink nlid="nl27" bibid="bib14" firstref="ref48"></nolink> <nolink nlid="nl28" bibid="bib25" firstref="ref49"></nolink> <nolink nlid="nl29" bibid="bib28" firstref="ref51"></nolink> <nolink nlid="nl30" bibid="bib26" firstref="ref63"></nolink>
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  Data: ADHD Symptoms and Insistence on Sameness in Prader-Willi Syndrome
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  Data: <searchLink fieldCode="AR" term="%22Wigren%2C+M%2E%22">Wigren, M.</searchLink><br /><searchLink fieldCode="AR" term="%22Hansen%2C+S%2E%22">Hansen, S.</searchLink>
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  Data: <searchLink fieldCode="SO" term="%22Journal+of+Intellectual+Disability+Research%22"><i>Journal of Intellectual Disability Research</i></searchLink>. Jun 2005 49(6):449-456.
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  Data: Journal Customer Services, Blackwell Publishing, 350 Main Street, Malden, MA 02148. Tel: 800-835-6770 (Toll Free); Fax: 781-388-8232; e-mail: subscrip@bos.blackwellpublishing.com.
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  Data: 8
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  Data: 2005
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  Data: Journal Articles<br />Reports - Research
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  Data: <searchLink fieldCode="DE" term="%22Profiles%22">Profiles</searchLink><br /><searchLink fieldCode="DE" term="%22Eating+Disorders%22">Eating Disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Symptoms+%28Individual+Disorders%29%22">Symptoms (Individual Disorders)</searchLink><br /><searchLink fieldCode="DE" term="%22Attention+Deficit+Disorders%22">Attention Deficit Disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Hyperactivity%22">Hyperactivity</searchLink><br /><searchLink fieldCode="DE" term="%22Disabilities%22">Disabilities</searchLink><br /><searchLink fieldCode="DE" term="%22Antisocial+Behavior%22">Antisocial Behavior</searchLink><br /><searchLink fieldCode="DE" term="%22Children%22">Children</searchLink><br /><searchLink fieldCode="DE" term="%22Adolescents%22">Adolescents</searchLink><br /><searchLink fieldCode="DE" term="%22Rating+Scales%22">Rating Scales</searchLink>
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  Data: 10.1111/j.1365-2788.2005.00690.x
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  Data: 0964-2633
– Name: Abstract
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  Data: Background: Apart from a pervasive eating disorder, the Prader-Willi (PWS) syndrome is characterized by a distinct behavioural profile comprising maladaptive behaviours, obsessive-compulsive traits and skin picking, all included in the PWS behavioural phenotype. In this study, we present a further delineation of this characteristic behavioural profile by screening for indices of executive dysfunctions related to attention-deficit hyperactivity disorder (ADHD), immature compulsive-like adherence to sameness and skin picking, and how these features aggregate into symptom constellations in children and adolescents with PWS. Method: Parents of 58 individuals with PWS (aged 5-18 years) participated by completing Childhood Routines Inventory (CRI) and Conners Parent Rating Scale (CPRS-48). Results: Results showed that indices of ADHD and excessive insistence on sameness were common, comorbid and of early onset. They were both associated with conduct problems. Skin picking, appearing as a single and comorbid symptom, was less associated with childlike compulsions and ADHD-related problems. Conclusions: Findings are discussed in terms of further research in executive dysfunctions in PWS.
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      – SubjectFull: Eating Disorders
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      – SubjectFull: Attention Deficit Disorders
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      – TitleFull: ADHD Symptoms and Insistence on Sameness in Prader-Willi Syndrome
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