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Dublin Core
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Title
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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
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<a href="http://doi.org/10.1111/1469-7610.00236" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/1469-7610.00236</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
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The Rett Syndrome Behaviour Questionnaire (RSBQ): refining the behavioural phenotype of Rett syndrome
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Journal of Child Psychology and Psychiatry
Date
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2002
Subject
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Psychology; children; adolescents; epidemiology; diagnosis; questionnaire; Rett syndrome; behavioural phenotype; Psychiatry; mecp2 mutations; intellectual disability; mental-retardation; cpg-binding protein-2; MECP2; mental retardation; population prevalence; psychopathology; x-chromosome inactivation; behavioral problems; breathing difficulties; sleep disturbance; tone and motor problems; tool development; scale development; RSBQ
Creator
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Mount R H; Charman T; Hastings R P; Reilly S; Cass H
Description
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Background: Although physical features, including loss of hand skills, deceleration of head growth, spasticity and scoliosis, are cardinal features of Rett syndrome (RS), a number of behavioural features are also associated with the disorder, including hand stereotypies, hyperventilation and breath holding. No study has tested the specificity of these behavioural features to individuals with RS, compared to individuals with severe to profound mental retardation (SMR). Method: A novel checklist of characteristic RS behavioural and emotional features, the Rett Syndrome Behaviour Questionnaire (RSBQ), was developed to test the type and specificity of behavioural features of RS against those found in girls with SMR. Results: After controlling for the effects of RS-related physical disabilities, the RSBQ discriminated between the groups. Some aspects of the behaviours found to be specific to RS are included in the necessary or supportive RS diagnostic criteria, notably hand behaviours and breathing problems. Additional behavioural features were also more frequently reported in the RS than the SMR group, including mood fluctuations and signs of fear/anxiety, inconsolable crying and screaming at night, and repetitive mouth and tongue movements and grimacing. Conclusions: Full validation of the scale requires confirmation of its discriminatory power and reliability with independent samples of individuals with RS and SMR. Further delineation of the specific profile of behaviours seen in RS may help in identification of the function of the MECP2 gene and in improved differential diagnosis and management of individuals with RS.
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<a href="http://doi.org/10.1111/1469-7610.00236" target="_blank" rel="noreferrer noopener">10.1111/1469-7610.00236</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2002
Adolescents
behavioral problems
behavioural phenotype
breathing difficulties
Cass H
Charman T
Children
cpg-binding protein-2
Diagnosis
Epidemiology
Hastings R P
Intellectual Disability
Journal Of Child Psychology And Psychiatry
mecp2
mecp2 mutations
Mental Retardation
mental-retardation
Mount R H
population prevalence
Psychiatry
Psychology
psychopathology
Questionnaire
Reilly S
Rett syndrome
RSBQ
scale development
sleep disturbance
tone and motor problems
tool development
x-chromosome inactivation