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Visual-Motor Integration in Children with Prader-Willi Syndrome

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Title: Visual-Motor Integration in Children with Prader-Willi Syndrome
Language: English
Authors: Lo, S. T., Collin, P. J. L., Hokken-Koelega, A. C. S.
Source: Journal of Intellectual Disability Research. Sep 2015 59(9):827-834.
Availability: Wiley-Blackwell. 350 Main Street, Malden, MA 02148. Tel: 800-835-6770; Tel: 781-388-8598; Fax: 781-388-8232; e-mail: cs-journals@wiley.com; Web site: http://www.wiley.com/WileyCDA
Peer Reviewed: Y
Page Count: 8
Publication Date: 2015
Document Type: Journal Articles
Reports - Research
Descriptors: Genetic Disorders, Mental Retardation, Perceptual Motor Coordination, Visual Perception, Children, Adolescents
Assessment and Survey Identifiers: Beery Developmental Test of Visual Motor Integration
DOI: 10.1111/jir.12197
ISSN: 0964-2633
Abstract: Background: Prader-Willi syndrome (PWS) is characterised by hypotonia, hypogonadism, short stature, obesity, behavioural problems, intellectual disability, and delay in language, social and motor development. There is very limited knowledge about visual-motor integration in children with PWS. Method: Seventy-three children with PWS aged 7-17 years were included. Visual-motor integration was assessed using the Beery Visual-motor Integration test at the start of the study and after 2 years. The association between visual-motor integration and age, gender, genetic subtype and intelligence was assessed. Results: Children with PWS scored "very low" (-3 standard deviations) in visual-motor integration and "below average" (-1 standard deviation) in visual perception and motor coordination compared with typically developing children. Visual-motor integration was higher in children with a deletion (ß?=?-0.170, P?=?0.037), in older children (ß?=?0.222, P?=?0.009) and in those with a higher total IQ (ß?=?0.784, P?
Abstractor: As Provided
Entry Date: 2015
Accession Number: EJ1072330
Database: ERIC
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  Value: <anid>AN0109016411;eul01sep.15;2018Jul09.15:24;v2.2.500</anid> <title id="AN0109016411-1">Visual-motor integration in children with Prader- Willi syndrome. </title> <p>Background: Prader–Willi syndrome (PWS) is characterised by hypotonia, hypogonadism, short stature, obesity, behavioural problems, intellectual disability, and delay in language, social and motor development. There is very limited knowledge about visual‐motor integration in children with PWS. Method: Seventy‐three children with PWS aged 7–17 years were included. Visual‐motor integration was assessed using the Beery Visual‐motor Integration test at the start of the study and after 2 years. The association between visual‐motor integration and age, gender, genetic subtype and intelligence was assessed. Results: Children with PWS scored ‘very low’ (−3 standard deviations) in visual‐motor integration and ‘below average’ (−1 standard deviation) in visual perception and motor coordination compared with typically developing children. Visual‐motor integration was higher in children with a deletion (β = −0.170, P = 0.037), in older children (β = 0.222, P = 0.009) and in those with a higher total IQ (β = 0.784, P < 0.001). Visual perception was higher with a deletion (β = −0.193, P = 0.044) and higher IQ (β = −0.618, P < 0.001), but motor coordination was only higher with a higher total IQ (β = 0.429, P = 0.001). Visual perception and motor coordination were not associated with age or gender. There was a trend for visual‐motor integration decline over the 2 year follow‐up period (P = 0.099). Visual perception and motor coordination did not change over the follow‐up period. Conclusions: Visual‐motor integration is very poor in children with PWS. Children scored higher on the time‐limited subtests for visual perception and motor coordination than the combined test for visual‐motor integration. Separation of visual‐motor integration tasks into pure visual or motor tasks and allowing sufficient time to perform the tasks might improve daily activities, both at home and at school.</p> <p>Prader–Willi syndrome; visual‐motor integration; visual perception; motor coordination</p> <p>Prader–Willi syndrome (PWS) is a neurogenetic developmental disorder due to the lack of expression of genes on the paternally inherited chromosome 15 at the locus q11‐q13, due to deletion (DEL), maternal uniparental disomy (mUPD), imprinting defects or chromosomal translocation (Cassidy [<reflink idref="bib8" id="ref1">8</reflink>] ). PWS is characterised by hypotonia, hypogonadism, short stature, obesity, behavioural problems, intellectual disability (ID) and developmental disabilities. Motor deficit is an important characteristic of PWS and is previously reported during pregnancy as lack in fetal activity by mothers and gynaecologists. After birth and during the first years of life, central hypotonia is the most marked characteristic. It causes decreased movements, a head lag, lethargy with decreased arousal, weak or absent cry, and poor reflexes, including a poor suck that leads to feeding difficulties and failure to thrive (Aughton & Cassidy [<reflink idref="bib2" id="ref2">2</reflink>] ). Also during infancy and childhood, impaired gross motor skills and fine motor skills are reported in children with PWS, resulting in delayed developmental milestones (Greenswag [<reflink idref="bib14" id="ref3">14</reflink>] ; Ehara et al. [<reflink idref="bib12" id="ref4">12</reflink>] ; Festen et al. [<reflink idref="bib13" id="ref5">13</reflink>] ; Reus et al. [<reflink idref="bib20" id="ref6">20</reflink>] ). Some studies investigated motor development and its relation to body composition and growth hormone treatment in children with PWS (Festen et al. [<reflink idref="bib13" id="ref7">13</reflink>] ; Carrel et al. [<reflink idref="bib6" id="ref8">6</reflink>] ; Reus et al. [<reflink idref="bib20" id="ref9">20</reflink>] ). However, knowledge about visual‐motor integration (VMI) capacity of children with PWS remained very limited.</p> <p>VMI shows the extent in which visual perception (interpretation of visual stimuli) and finger‐hand movement are coordinated (Beery & Beery [<reflink idref="bib4" id="ref10">4</reflink>] ). VMI is an important contributor to academic skills that require eye–hand coordination, such as writing, mathematical formulations and drawing. It requires accurate perception of visual spatial objects and monitoring of one's own movement. VMI skills are thus embedded in daily activities of school‐age children. Insight into VMI in childhood is therefore clinically relevant to optimise the approach and guidance of daily activities at school in children with PWS. To our knowledge, VMI in PWS has only been studied in two studies using the Beery‐Buktenica Developmental Test of Visual‐motor Integration (Beery‐VMI). The first research group concluded that VMI was more impaired in children and adolescents with PWS than age‐matched typically developing youngsters (Dykens [<reflink idref="bib10" id="ref11">10</reflink>] ). The second research group found that individuals with the smaller type II DEL performed better on visual processing than those with the larger type I DEL or mUPD (Butler et al. [<reflink idref="bib5" id="ref12">5</reflink>] ). Both studies were performed in a relatively small group of individuals with PWS (21 and 12, respectively) and no separate assessment of visual processing and motor coordination was performed. The present study was conducted to investigate the level of VMI in more detail in a large group of children with PWS.</p> <p>The IQ range in individuals with PWS varies between 50 and 80 (Milner et al. [<reflink idref="bib18" id="ref13">18</reflink>] ; Lo et al. [<reflink idref="bib16" id="ref14">16</reflink>] ). VMI testing in 5‐year‐old children with 22q11 deletion syndrome, who have a similar IQ as children with PWS, showed that VMI performance was below average compared with typically developing peers (Duijff et al. [<reflink idref="bib9" id="ref15">9</reflink>] ). In children with 22q11 deletion syndrome, VMI and visual skills were correlated with total IQ, but not with motor coordination.</p> <p>Earlier studies have found children with PWS to be stronger in performing visual spatial tasks than their peers with similar IQ (Dykens [<reflink idref="bib10" id="ref16">10</reflink>] ), with individuals with a DEL scoring higher on the VMI than those with an mUPD (Dykens [<reflink idref="bib10" id="ref17">10</reflink>] ; Veltman et al. [<reflink idref="bib23" id="ref18">23</reflink>] ). However, these studies were performed in a group with a wide age range, or in instances where it was not known if the participants were treated with growth hormone.</p> <p>In our previous study, we found that visual processing deteriorated during 2 years in children without growth hormone treatment, but improved significantly after long‐term growth hormone treatment, especially in those with a greater deficit (Siemensma et al. [<reflink idref="bib21" id="ref19">21</reflink>] ). As growth hormone treatment is now a standard treatment in most developed countries, this might suggest that long‐term growth hormone treatment not only improves visual processing, but might also improve VMI.</p> <p>The aim of our study was to determine the level of VMI. Secondly, we investigated which factors predicted the level of VMI in children with PWS. We hypothesised that VMI in children with PWS is impaired compared with healthy references, but that children with DEL score higher than those with mUPD. In addition, we expected that VMI performance and visual perception would correlate with total IQ, and motor coordination with performal IQ, and that the level of VMI would decline over time in children with PWS compared with healthy references.</p> <hd id="AN0109016411-2">Methods</hd> <p>For the present study, we enrolled children aged 7–17 years who participated in the ongoing Dutch PWS Cohort study, a study investigating the long‐term effects of growth hormone treatment in children with PWS (Bakker et al. [<reflink idref="bib3" id="ref20">3</reflink>] ). Testing for VMI was performed at baseline and after 2 years.</p> <p>A total of 81 children with PWS were eligible for participation, 75 of whom agreed to participate, resulting in a response rate of 93%. In the second assessment, 54 children who also completed the first assessment were eligible to participate, and 52 children completed the second assessment, resulting in a response rate of 96%. Twenty‐one children were excluded from the second assessment due to the follow‐up period being less than 2 years, one died, and one child did not participate.</p> <p>The genetic diagnosis of PWS was confirmed in all participants by methylation testing. The genetic subtype was known in all but four participants. All children were treated with growth hormone (Genotropin 1 mg/m<sups>2</sups>/day). This study was approved by the Medical Ethics Committee of the Erasmus University Medical Center in Rotterdam. Written informed consent was obtained from all parents or caretakers and children of 12 years of age onwards, and assent below the age of 12 years.</p> <hd id="AN0109016411-3">Visual‐motor integration</hd> <p>VMI was tested with the Beery‐VMI (full form), which is designed to assess to which extent individuals can integrate their visual and motor abilities by copying geometric forms, organised in a developmental sequence. The Beery‐VMI is valid for individuals aged 2–100 years to assess the written visual‐motor functioning (Beery & Beery [<reflink idref="bib4" id="ref21">4</reflink>] ). The child was asked to copy 30 geometric forms, organised from simple to more complicated forms. No time limit was required for this test. VMI has been investigated in 5‐year‐old children with 22q11 deletion syndrome and small groups of children with PWS, confirming its feasibility (Dykens [<reflink idref="bib10" id="ref22">10</reflink>] ; Butler et al. [<reflink idref="bib5" id="ref23">5</reflink>] ; Duijff et al. [<reflink idref="bib9" id="ref24">9</reflink>] ).</p> <hd id="AN0109016411-4">Visual perception</hd> <p>The VMI Supplemental Test for Visual Perception is a test to assess visual performance by reducing motor requirements of the task to a minimum (Beery & Beery [<reflink idref="bib4" id="ref25">4</reflink>] ). A geometric form was presented to the child and we asked the child to point to the exact same geometric form within a group of other forms, organised from simple to more complicated. The testing time was limited to 3 min for 30 items.</p> <hd id="AN0109016411-5">Motor coordination</hd> <p>The VMI Supplemental Test for Motor Coordination is a test to assess motor performance by reducing visual perceptual demands to a minimum (Beery & Beery [<reflink idref="bib4" id="ref26">4</reflink>] ). The child was asked to trace the stimulus forms with a pencil, staying between the double lined paths. Visual perception was minimised in this subtest by providing starting dots and paths as strong visual guides for the required motor performance. The test consists of 30 items ranging from simple to more complicated forms. All of the geometric forms were the same as the geometric figures used in the Beery‐VMI. The testing time was limited to 5 min for 30 items.</p> <p>Only pencil and paper were allowed for all three tests. No corrections were allowed. All tests were only performed if the child could complete the three example forms after a brief description of the particular task. All three tests consisted of the same 30 geometric figures. The Beery‐VMI is well validated and has a sound reliability of 0.92 for VMI, 0.91 for visual perception and 0.90 for motor coordination (Beery & Beery [<reflink idref="bib4" id="ref27">4</reflink>] ).</p> <hd id="AN0109016411-6">Cognitive functioning</hd> <p>Cognitive testing was annually performed in prepubertal children and biennially in pubertal children using the short form of the Wechsler Intelligence Scale for Children‐Revised (Wechsler [<reflink idref="bib24" id="ref28">24</reflink>] ). All cognitive measurements described in this study were performed by an experienced psychologist. The methods of cognitive testing in prepubertal children have been described in detail (Lo et al. [<reflink idref="bib16" id="ref29">16</reflink>] ). In summary, total IQ score was calculated according to an equation based on a Dutch outpatient reference population (total IQ = 45.3 + 2.91 × vocabulary s‐score + 2.50 × block design s‐score), as used in other studies (van Pareren et al. [<reflink idref="bib19" id="ref30">19</reflink>] ; Hokken‐Koelega et al. [<reflink idref="bib15" id="ref31">15</reflink>] ; Siemensma et al. [<reflink idref="bib21" id="ref32">21</reflink>] ). The vocabulary subtest was used to assess verbal IQ, and the block design subtest was used for performal IQ.</p> <hd id="AN0109016411-7">Data analysis</hd> <p>Age and intelligence at baseline are presented as median and interquartile range (IQR). Results for VMI, visual perception and motor coordination are expressed as a standard score provided by the manual of the Beery‐VMI according to an individual's age. Standard scores consist of equal units of measurement with a mean of 100 and a standard deviation of 15. The standard score of performance was compared with a healthy reference group included in the manual, i.e. ‘average’ performance (standard score 90–109, includes 50% of the age group, equals 0 standard deviation), ‘below average’ (standard score 80–89, 16% of the age group, equals −1 standard deviation), ‘low’ (standard score 70–79, 7% of the age group, equals −2 standard deviation), ‘very low’ (standard score <70, 2% of the age group, equals −3 standard deviation) (Beery & Beery [<reflink idref="bib4" id="ref33">4</reflink>] ). The equivalent developmental age to the raw score was calculated using the Beery VMI Raw Score Age Equivalents table (Beery & Beery [<reflink idref="bib4" id="ref34">4</reflink>] ). Multiple linear regression was performed to investigate the association between VMI and age, gender (1 = boys, 2 = girls), genetic subtype (1 = DEL, 2 = mUPD), total IQ, verbal IQ and performal IQ. McNemar's test was used to test for differences in gender and genetic subtype at baseline and after 2 years. Wilcoxon signed ranks test was used to test for differences in total IQ and standard scores for VMI, visual perception and motor coordination between the group at start and after 2 years. Statistical analyses were performed with SPSS 20.0 (SPSS Inc., Chicago, IL, USA). A P‐value of <0.05 (two tailed) was considered statistically significant.</p> <hd id="AN0109016411-8">Results</hd> <hd id="AN0109016411-9">Baseline</hd> <p>The median IQR age of the 75 children with PWS was 11.8 (8.7, 14.1) years and 34 (45%) were male (Table [NaN] ). Thirty‐one (41%) had DEL, 36 (48%) mUPD, and 4 had an imprinting defect, and in four children the genetic subtype remained unknown. Median (IQR) total IQ was 62 (<reflink idref="bib56" id="ref35">56</reflink>, 78), age at start of growth hormone treatment 4.5 (2.2, 6.8) years, and duration of growth hormone treatment 7.0 (6.0, 8.0) years. The characteristics of the study group are presented in Table [NaN] .</p> <p>Characteristics of the study group at baseline</p> <p> <ephtml> <table><tr><th /><th>Baseline</th></tr><tr><td>N (male)</td><td>75 (34)</td></tr><tr><td>Age (years)</td><td>11.8 (8.7, 14.1)</td></tr><tr><td>Genetic subtype [n (%)]</td><td /></tr><tr><td>Deletion</td><td>31 (41%)</td></tr><tr><td>UPD</td><td>36 (48%)</td></tr><tr><td>ICD</td><td>4</td></tr><tr><td>Unknown</td><td>4</td></tr><tr><td>Total IQ</td><td>62 (56, 78)</td></tr><tr><td>Age at start of GH treatment (years)</td><td>4.5 (2.2, 6.8)</td></tr><tr><td>Duration of GH treatment (years)</td><td>7.0 (6.0, 8.0)</td></tr></table> </ephtml> </p> <p>1 Age, total IQ, age at start and duration of GH treatment are expressed in median (IQR).</p> <p>2 GH, growth hormone.</p> <hd id="AN0109016411-10">Visual‐motor integration</hd> <p>Children with PWS scored ‘very low’ (−3 standard deviations) in VMI with a median (IQR) standard score of 62 (<reflink idref="bib45" id="ref36">45</reflink>, 81). They scored ‘below average’ (−1 standard deviation) in visual perception and motor coordination with median scores of 81 (<reflink idref="bib62" id="ref37">62</reflink>, 89) and 80 (<reflink idref="bib63" id="ref38">63</reflink>, 93), respectively The VMI standard scores of individual children with PWS according to age are presented in Fig. [NaN] and the age equivalent to the VMI standard score according to age in Fig. [NaN] .</p> <p>Multiple linear regression showed that VMI was higher in children with a DEL (β = −0.170, P = 0.037), in older children (β = 0.222, P = 0.009) and in those with a higher total IQ (β = 0.784, P < 0.001) (Table [NaN] ), but there was no difference in VMI between boys and girls (β = 0.065, P = 0.418). Higher verbal IQ (β = 0.419, P < 0.001) and performal IQ (β = 0.469, P < 0.001) were both associated with higher VMI performance. Visual perception and motor coordination were higher in children with a higher total IQ (β = 0.618, P < 0.001 and β = 0.429, P = 0.001, respectively). Visual perception was higher in children with higher verbal (β = 0.339, P = 0.005) and performal IQs (β = 0.398, P = 0.003), but there was no association between motor coordination and verbal (β = 0.252, P = 0.095) or performal IQ (β = 0.236, P = 0.146). Visual perception was also higher in children with a DEL (β = −0.193, P = 0.044).</p> <p>VMI in children with PWS</p> <p> <ephtml> <table><tr><th /><th>VMI</th><th>Visual perception</th><th>Motor coordination</th></tr><tr><th>Model A</th><th>Model B</th><th>Model A</th><th>Model B</th><th>Model A</th><th>Model B</th></tr><tr><th>ß</th><th>P</th><th>ß</th><th>P</th><th>ß</th><th>P</th><th>ß</th><th>P</th><th>ß</th><th>P</th><th>ß</th><th>P</th></tr><tr><td>Age</td><td>0.222</td><td>0.009</td><td>0.270</td><td>0.004</td><td>−0.082</td><td>0.404</td><td>−0.055</td><td>0.605</td><td>−0.038</td><td>0.753</td><td>−0.005</td><td>0.970</td></tr><tr><td>DEL vs. mUPD</td><td>−0.170</td><td>0.037</td><td>−0.164</td><td>0.054</td><td>−0.193</td><td>0.044</td><td>−0.157</td><td>0.112</td><td>−0.059</td><td>0.615</td><td>−0.038</td><td>0.757</td></tr><tr><td>Total IQ</td><td>0.784</td><td><0.001</td><td /><td /><td>0.618</td><td><0.001</td><td /><td /><td>0.429</td><td>0.001</td><td /><td /></tr><tr><td>Verbal IQ</td><td /><td /><td>0.419</td><td><0.001</td><td /><td /><td>0.339</td><td>0.005</td><td /><td /><td>0.252</td><td>0.095</td></tr><tr><td>Performal IQ</td><td /><td /><td>0.469</td><td><0.001</td><td /><td /><td>0.398</td><td>0.003</td><td /><td /><td>0.236</td><td>0.146</td></tr><tr><td>Overall P‐value</td><td><0.001</td><td><0.001</td><td><0.001</td><td><0.001</td><td>0.004</td><td>0.021</td></tr><tr><td>Adj. R<sup>2</sup></td><td>0.602</td><td>0.598</td><td>0.452</td><td>0.456</td><td>0.173</td><td>0.140</td></tr></table> </ephtml> </p> <p>3 VMI, visual perception and motor coordination are expressed in standard scores. Genetic subtype is classified as follows: DEL = 1, mUPD = 2. Adjusted for gender. The bold numbers are indicating which p‐values are significant.</p> <hd id="AN0109016411-11">After 2 years of follow‐up</hd> <p>In the second assessment after 2 years, 52 children participated and this group did not significantly differ from the initial group in gender, genetic subtype and total IQ (P = 0.317, P = 1.000 and P = 0.203, respectively).</p> <p>There was a trend for VMI decline over the 2 year follow‐up (z = −1.651, P = 0.099). There was no change in visual perception (z = −0.233, P = 0.815) and motor coordination (z = −0.622, P = 0.534) after 2 years of follow‐up.</p> <hd id="AN0109016411-12">Discussion</hd> <p>Our study shows that VMI in children with PWS is very poor compared with typically developing children. The majority of our group had a standard deviation score of less than −3. VMI was higher in children with a deletion, in older children and in those with a higher total IQ. However, there was no difference in VMI between boys and girls. Children scored higher when visual perception and motor coordination were tested separately. Visual perception and motor coordination were positively associated with total IQ, but only VMI and visual perception were positively associated with verbal and performal IQs. During the 2 year follow‐up, the visual perception and motor coordination did not change, but there was a trend for VMI to decline.</p> <p>VMI is the ability to integrate visual perception and motor skills. We tested VMI by asking children to copy a figure, for which both visual perception and motor coordination were required. Visual perception and motor coordination were also tested separately to determine if these skills were impaired on their own. As expected, VMI was considerably impaired in our study group which resulted in a −3 standard deviation score compared with typically developing children. Unexpectedly, the children had lower scores in the no time limit performance of the Beery‐VMI than in the other time‐limited subtests. As Beery‐VMI tests the integration of visual perception and motor coordination, the results suggest that VMI is more difficult for children with PWS than performing a simplified task, either visual or motor, even in the case of time restriction. This poor VMI performance explains at least part of the difficulties in daily functioning faced by people with PWS, such as the weakness in writing skills. Based on the results of our study, writing could be better taught by training the visual and motor skills separately. For example, the visual differences between letters could be trained first followed by motor tasks. The actual writing of letters should be the last step of the learning process as VMI is the most difficult step for these children. It is likely that poor VMI skills also contribute to difficulties in other motor coordination skills, e.g. getting dressed. For daily practice, visual support could be provided by using pictograms.</p> <p>The majority of children were eager to finish the tests beyond the permitted time. In our analyses, we only used the scores within the time limit in order to compare the results with typically developing peers. But observationally, children were able to score higher on the visual and motor tests if there was no time limit. Future studies on VMI in children with PWS could investigate their maximal ability by recording both scores with limited and unlimited time settings for the visual and motor tests. Hypotonia, a typical feature of PWS, might partly explain why individuals with PWS need more time to complete a motor task.</p> <p>We expected that VMI would decline over time and thus that the difference in VMI skills would increase between children with PWS and their typically developing peers. We found, however, that there was a trend in VMI decline, as this was at the border of significance. It is most likely that the time period of 2 years was too short to show any difference in individual VMI performance.</p> <p>VMI performance was strongly associated with total IQ, which is in line with an earlier report in children with ID (Memisevic & Sinanovic [<reflink idref="bib17" id="ref39">17</reflink>] ). This study suggested that intelligence might be particularly related to the planning stage of the VMI task and not so much to the motor component of the task. Hypothetically, altered sensory perception due to derangement of neurotransmitter balance involving the hypothalamic regions could have delayed the processing speed in individuals with PWS (Akefeldt et al. [<reflink idref="bib1" id="ref40">1</reflink>] ). In our study group, two boys with an mUPD of 15 and 17 years old scored unexpectedly higher on VMI than their peers with the same genetic subtype. Both boys started growth hormone treatment around the age of 9 years, were treated for 6–7 years, and had a total IQ of 86 and 90, respectively. Their IQs were in the highest 10% of the total study group.</p> <p>We did not find a difference in VMI performance between boys and girls, which is in contrast to a previous study in children with ID reporting that girls performed better than boys (Memisevic & Sinanovic [<reflink idref="bib17" id="ref41">17</reflink>] ). However, another study in children with 22q11 deletion also found no difference in VMI performance between boys and girls (Duijff et al. [<reflink idref="bib9" id="ref42">9</reflink>] ).</p> <p>In our study group, 41% of children had a deletion and 48% had an mUPD. Earlier studies reported a DEL : mUPD ratio of 70:30 (Cassidy [<reflink idref="bib7" id="ref43">7</reflink>] ; Dykens & Roof [<reflink idref="bib11" id="ref44">11</reflink>] ). The higher frequency of the mUPD type in our Dutch study group is most likely related to an increased maternal age, which is in line with previous studies in Western Europe (Sinnema et al. [<reflink idref="bib22" id="ref45">22</reflink>] ; Whittington et al. [<reflink idref="bib25" id="ref46">25</reflink>] ).</p> <p>As the purpose of this study was to investigate the level of VMI in a large group of children with PWS, we did not include a comparison group with children with ID. Our study, therefore, does not differentiate which characteristics are ‘Prader–Willi specific’. However, children with DEL scored higher on VMI and visual perception than those with mUPD, which is in line with an earlier finding that children with DEL have stronger visual skills (Dykens [<reflink idref="bib10" id="ref47">10</reflink>] ; Veltman et al. [<reflink idref="bib23" id="ref48">23</reflink>] ). Earlier studies showed a beneficial effect of growth hormone treatment on motor skills and IQ (Festen et al. [<reflink idref="bib13" id="ref49">13</reflink>] ; Carrel et al. [<reflink idref="bib6" id="ref50">6</reflink>] ; Siemensma et al. [<reflink idref="bib21" id="ref51">21</reflink>] ; Reus et al. [<reflink idref="bib20" id="ref52">20</reflink>] ). However, our study was not designed to investigate the effect of growth hormone treatment on VMI.</p> <p>In conclusion, VMI performance was very poor (−3 standard deviations) in a large group of 73 children with PWS compared with healthy references. VMI was higher in children with a deletion, in older children and in those with a higher total IQ. Separation of VMI tasks into pure visual or motor tasks and allowing sufficient time to perform the tasks might improve daily school activities such as writing. Our findings provide further insight into the neurocognitive deficiencies in children with PWS and how to approach these.</p> <hd id="AN0109016411-13">Acknowledgments</hd> <p>We express our gratitude to all children and parents for their enthusiastic participation in this study and acknowledge the work of P.M.C.C. van Eekelen, research nurse, and E. Mahabier‐Janssen, psychologist. This study was supported by the Dutch Prader‐Willi Fund, Fund NutsOhra and the Dutch Growth Research Foundation.</p> <ref id="AN0109016411-14"> <title>References</title> <blist> <bibl id="bib1" idref="ref40" type="bt">1</bibl> <bibtext>Akefeldt A., Ekman R., Gillberg C. & Mansson J. E. ( 1998 ) Cerebrospinal fluid monoamines in Prader‐Willi syndrome. Biological Psychiatry 44, 1321 – 1328. </bibtext> </blist> <blist> <bibl id="bib2" idref="ref2" type="bt">2</bibl> <bibtext>Aughton D. J. & Cassidy S. B. ( 1990 ) Physical features of Prader‐Willi syndrome in neonates. American Journal of Diseases of Children 144, 1251 – 1254. </bibtext> </blist> <blist> <bibl id="bib3" idref="ref20" type="bt">3</bibl> <bibtext>Bakker N. E., Kuppens R. J., Siemensma E. P., Tummers‐de Lind van Wijngaarden R. F., Festen D. A., Bindels‐de Heus G. C. et al. ( 2013 ) Eight years of growth hormone treatment in children with Prader‐Willi syndrome: maintaining the positive effects. Journal of Clinical Endocrinology and Metabolism 98, 4013 – 4022. </bibtext> </blist> <blist> <bibl id="bib4" idref="ref10" type="bt">4</bibl> <bibtext>Beery K. E. & Beery N. A. ( 2004 ) The Beery‐Buktenica Developmental Test of Visual‐Motor Integration: Beery VMI with Supplemental Developmental Tests of Visual Perception and Motor Coordination: Administration, Scoring and Teaching Manual, 6th edn. NCS Pearson Inc, Minneapolis, MN. </bibtext> </blist> <blist> <bibl id="bib5" idref="ref12" type="bt">5</bibl> <bibtext>Butler M. G., Bittel D. C., Kibiryeva N., Talebizadeh Z. & Thompson T. ( 2004 ) Behavioral differences among subjects with Prader‐Willi syndrome and type I or type II deletion and maternal disomy. Pediatrics 113 ( 3 Pt 1 ), 565 – 573. </bibtext> </blist> <blist> <bibl id="bib6" idref="ref8" type="bt">6</bibl> <bibtext>Carrel A. L., Myers S. E., Whitman B. Y., Eickhoff J. & Allen D. B. ( 2010 ) Long‐term growth hormone therapy changes the natural history of body composition and motor function in children with Prader‐Willi syndrome. Journal of Clinical Endocrinology and Metabolism 95, 1131 – 1136. </bibtext> </blist> <blist> <bibl id="bib7" idref="ref43" type="bt">7</bibl> <bibtext>Cassidy S. B. ( 1984 ) Prader‐Willi syndrome. Current Problems in Pediatrics 14, 1 – 55. </bibtext> </blist> <blist> <bibl id="bib8" idref="ref1" type="bt">8</bibl> <bibtext>Cassidy S. B. ( 1997 ) Prader‐Willi syndrome. Journal of Medical Genetics 34, 917 – 923. </bibtext> </blist> <blist> <bibl id="bib9" idref="ref15" type="bt">9</bibl> <bibtext>Duijff S., Klaassen P., Beemer F., Swanenburg de Veye H., Vorstman J. & Sinnema G. ( 2012 ) Intelligence and visual‐motor integration in 5‐year‐old children with 22q11‐deletion syndrome. Research in Developmental Disabilities 33, 334 – 340. </bibtext> </blist> <blist> <bibl id="bib10" idref="ref11" type="bt">10</bibl> <bibtext>Dykens E. M. ( 2002 ) Are jigsaw puzzle skills ‘spared’ in persons with Prader‐Willi syndrome? Journal of Child Psychology and Psychiatry, and Allied Disciplines 43, 343 – 352. </bibtext> </blist> <blist> <bibl id="bib11" idref="ref44" type="bt">11</bibl> <bibtext>Dykens E. M. & Roof E. ( 2008 ) Behavior in Prader‐Willi syndrome: relationship to genetic subtypes and age. Journal of Child Psychology and Psychiatry, and Allied Disciplines 49, 1001 – 1008. </bibtext> </blist> <blist> <bibl id="bib12" idref="ref4" type="bt">12</bibl> <bibtext>Ehara H., Ohno K. & Takeshita K. ( 1993 ) Growth and developmental patterns in Prader‐Willi syndrome. Journal of Intellectual Disability Research 37 ( Pt 5 ), 479 – 485. </bibtext> </blist> <blist> <bibl id="bib13" idref="ref5" type="bt">13</bibl> <bibtext>Festen D. A., Wevers M., Lindgren A. C., Bohm B., Otten B. J., Wit J. M. et al. ( 2008 ) Mental and motor development before and during growth hormone treatment in infants and toddlers with Prader‐Willi syndrome. Clinical Endocrinology 68, 919 – 925. </bibtext> </blist> <blist> <bibl id="bib14" idref="ref3" type="bt">14</bibl> <bibtext>Greenswag L. R. ( 1987 ) Adults with Prader‐Willi syndrome: a survey of 232 cases. Developmental Medicine and Child Neurology 29, 145 – 152. </bibtext> </blist> <blist> <bibl id="bib15" idref="ref31" type="bt">15</bibl> <bibtext>Hokken‐Koelega A. C., van Pareren Y. K. & Arends N. ( 2005 ) Effects of growth hormone treatment on cognitive function and head circumference in children born small for gestational age. Hormone Research 64 ( Suppl. 3 ), 95 – 99. </bibtext> </blist> <blist> <bibl id="bib16" idref="ref14" type="bt">16</bibl> <bibtext>Lo S. T., Siemensma E., Collin P. & Hokken‐Koelega A. ( 2013 ) Impaired theory of mind and symptoms of Autism Spectrum Disorder in children with Prader‐Willi syndrome. Research in Developmental Disabilities 34, 2764 – 2773. </bibtext> </blist> <blist> <bibl id="bib17" idref="ref39" type="bt">17</bibl> <bibtext>Memisevic H. & Sinanovic O. ( 2012 ) Predictors of visual‐motor integration in children with intellectual disability. International Journal of Rehabilitation Research 35, 372 – 374. </bibtext> </blist> <blist> <bibl id="bib18" idref="ref13" type="bt">18</bibl> <bibtext>Milner K. M., Craig E. E., Thompson R. J., Veltman M. W., Thomas N. S., Roberts S. et al. ( 2005 ) Prader‐Willi syndrome: intellectual abilities and behavioural features by genetic subtype. Journal of Child Psychology and Psychiatry 46, 1089 – 1096. </bibtext> </blist> <blist> <bibl id="bib19" idref="ref30" type="bt">19</bibl> <bibtext>van Pareren Y. K., Duivenvoorden H. J., Slijper F. S., Koot H. M. & Hokken‐Koelega A. C. ( 2004 ) Intelligence and psychosocial functioning during long‐term growth hormone therapy in children born small for gestational age. Journal of Clinical Endocrinology and Metabolism 89, 5295 – 5302. </bibtext> </blist> <blist> <bibl id="bib20" idref="ref6" type="bt">20</bibl> <bibtext>Reus L., Pelzer B. J., Otten B. J., Siemensma E. P., van Alfen‐van der Velden J. A., Festen D. A. et al. ( 2013 ) Growth hormone combined with child‐specific motor training improves motor development in infants with Prader‐Willi syndrome: a randomized controlled trial. Research in Developmental Disabilities 34, 3092 – 3103. </bibtext> </blist> <blist> <bibl id="bib21" idref="ref19" type="bt">21</bibl> <bibtext>Siemensma E. P., Tummers‐de Lind van Wijngaarden R. F., Festen D. A., Troeman Z. C., van Alfen‐van der Velden A. A., Otten B. J. et al. ( 2012 ) Beneficial effects of growth hormone treatment on cognition in children with Prader‐Willi syndrome: a randomized controlled trial and longitudinal study. Journal of Clinical Endocrinology and Metabolism 97, 2307 – 2314. </bibtext> </blist> <blist> <bibl id="bib22" idref="ref45" type="bt">22</bibl> <bibtext>Sinnema M., Boer H., Collin P., Maaskant M. A., van Roozendaal K. E., Schrander‐Stumpel C. T. et al. ( 2011 ) Psychiatric illness in a cohort of adults with Prader‐Willi syndrome. Research in Developmental Disabilities 32, 1729 – 1735. </bibtext> </blist> <blist> <bibl id="bib23" idref="ref18" type="bt">23</bibl> <bibtext>Veltman M. W., Thompson R. J., Roberts S. E., Thomas N. S., Whittington J. & Bolton P. F. ( 2004 ) Prader‐Willi syndrome – a study comparing deletion and uniparental disomy cases with reference to autism spectrum disorders. European Child and Adolescent Psychiatry 13, 42 – 50. </bibtext> </blist> <blist> <bibl id="bib24" idref="ref28" type="bt">24</bibl> <bibtext>Wechsler D. ( 2002 ) Wechsler Intelligence Scale for Children (Dutch Version), Manual, 3rd edn. Harcourt Assessment, London, UK. </bibtext> </blist> <blist> <bibl id="bib25" idref="ref46" type="bt">25</bibl> <bibtext>Whittington J. E., Butler J. V. & Holland A. J. ( 2007 ) Changing rates of genetic subtypes of Prader‐Willi syndrome in the UK. European Journal of Human Genetics 15, 127 – 130. </bibtext> </blist> </ref> <p>Graph: VMI performance in children with PWS.</p> <p>Graph: VMI performance expressed as age equivalent in children with PWS. SS, standard score. The line of identity (45°) represents the line of 0 standard deviation (SD) and is similar to age. The line below represents the −2 SD line for typically developing children.</p> <aug> <p>By S. T. Lo; P. J. L. Collin and A. C. S. Hokken‐Koelega</p> </aug> <nolink nlid="nl1" bibid="bib56" firstref="ref35"></nolink> <nolink nlid="nl2" bibid="bib45" firstref="ref36"></nolink> <nolink nlid="nl3" bibid="bib62" firstref="ref37"></nolink> <nolink nlid="nl4" bibid="bib63" firstref="ref38"></nolink>
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  Data: Visual-Motor Integration in Children with Prader-Willi Syndrome
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  Data: <searchLink fieldCode="AR" term="%22Lo%2C+S%2E+T%2E%22">Lo, S. T.</searchLink><br /><searchLink fieldCode="AR" term="%22Collin%2C+P%2E+J%2E+L%2E%22">Collin, P. J. L.</searchLink><br /><searchLink fieldCode="AR" term="%22Hokken-Koelega%2C+A%2E+C%2E+S%2E%22">Hokken-Koelega, A. C. 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>. Sep 2015 59(9):827-834.
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  Data: Wiley-Blackwell. 350 Main Street, Malden, MA 02148. Tel: 800-835-6770; Tel: 781-388-8598; Fax: 781-388-8232; e-mail: cs-journals@wiley.com; Web site: http://www.wiley.com/WileyCDA
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  Data: 8
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  Data: <searchLink fieldCode="DE" term="%22Genetic+Disorders%22">Genetic Disorders</searchLink><br /><searchLink fieldCode="DE" term="%22Mental+Retardation%22">Mental Retardation</searchLink><br /><searchLink fieldCode="DE" term="%22Perceptual+Motor+Coordination%22">Perceptual Motor Coordination</searchLink><br /><searchLink fieldCode="DE" term="%22Visual+Perception%22">Visual Perception</searchLink><br /><searchLink fieldCode="DE" term="%22Children%22">Children</searchLink><br /><searchLink fieldCode="DE" term="%22Adolescents%22">Adolescents</searchLink>
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  Data: 10.1111/jir.12197
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  Data: 0964-2633
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  Data: Background: Prader-Willi syndrome (PWS) is characterised by hypotonia, hypogonadism, short stature, obesity, behavioural problems, intellectual disability, and delay in language, social and motor development. There is very limited knowledge about visual-motor integration in children with PWS. Method: Seventy-three children with PWS aged 7-17 years were included. Visual-motor integration was assessed using the Beery Visual-motor Integration test at the start of the study and after 2 years. The association between visual-motor integration and age, gender, genetic subtype and intelligence was assessed. Results: Children with PWS scored "very low" (-3 standard deviations) in visual-motor integration and "below average" (-1 standard deviation) in visual perception and motor coordination compared with typically developing children. Visual-motor integration was higher in children with a deletion (ß?=?-0.170, P?=?0.037), in older children (ß?=?0.222, P?=?0.009) and in those with a higher total IQ (ß?=?0.784, P?<?0.001). Visual perception was higher with a deletion (ß?=?-0.193, P?=?0.044) and higher IQ (ß?=?-0.618, P?<?0.001), but motor coordination was only higher with a higher total IQ (ß?=?0.429, P?=?0.001). Visual perception and motor coordination were not associated with age or gender. There was a trend for visual-motor integration decline over the 2 year follow-up period (P?=?0.099). Visual perception and motor coordination did not change over the follow-up period. Conclusions: Visual-motor integration is very poor in children with PWS. Children scored higher on the time-limited subtests for visual perception and motor coordination than the combined test for visual-motor integration. Separation of visual-motor integration tasks into pure visual or motor tasks and allowing sufficient time to perform the tasks might improve daily activities, both at home and at school.
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      – SubjectFull: Mental Retardation
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      – SubjectFull: Perceptual Motor Coordination
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      – SubjectFull: Beery Developmental Test of Visual Motor Integration
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      – TitleFull: Visual-Motor Integration in Children with Prader-Willi Syndrome
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