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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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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.ejpn.2011.12.014" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ejpn.2011.12.014</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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Beyond the Burke-Fahn-Marsden Dystonia Rating Scale: Deep brain stimulation in childhood secondary dystonia
Publisher
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European Journal of Paediatric Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2012
Subject
The topic of the resource
children; Pediatrics; Goals; Disability; reliability; Outcomes; patient selection; validity; Neurosciences & Neurology; cerebral-palsy; follow-up; rehabilitation; globus-pallidus internus; primary generalized dystonia; (DBS); Childhood dystonia; Paediatric deep brain stimulation; pediatric movement-disorders; Secondary dystonia; tone and motor problems; Glutaric acidemia type I; surgical intervention; Deep brain stimulation; BFMDRS
Creator
An entity primarily responsible for making the resource
Gimeno H; Tustin K; Selway R; Lin J P
Description
An account of the resource
Purpose: Deep brain stimulation is now widely accepted as an effective treatment for children with primary generalized dystonia. More variable results are reported in secondary dystonias and its efficacy in this heterogeneous group has not been fully elucidated. Deep brain stimulation outcomes are typically reported using impairment-focused measures, such as the Burke-Fahn-Marsden Dystonia Rating Scale, which provide little information about function and participation outcomes or changes in non-motor areas. The aim is to demonstrate that in some cases of secondary dystonia, the sole use of impairment level measures, such as the Burke-Fahn-Marsden Dystonia Rating Scale, may be insufficient to fully evaluate outcome following deep brain stimulation. Methods: Six paediatric cases who underwent deep brain stimulation surgery with a minimum of one year follow up were selected on the basis of apparent non-response to deep brain stimulation, defined as a clinically insignificant change in the Burke-Fahn-Marsden Dystonia Movement Scale (<20%), but where other evaluation measures demonstrated clinical efficacy across several domains. Results: Despite no significant change in Burke-Fahn-Marsden Dystonia Rating Scale scores following deep brain stimulation, parallel outcome measures demonstrated significant benefit in a range of child and family-centred goal areas including: pain and comfort, school attendance, seating tolerance, access to assistive technology and in some cases carer burden. Conclusions: Sole use of impairment-focused measures, are limited in scope to evaluate outcome following deep brain stimulation, particularly in secondary dystonias. Systematic study of effects across multiple dimensions of disability is needed to determine what deep brain stimulation offers patients in terms of function, participation, care, comfort and quality of life. Deep brain stimulation may offer meaningful change across multiple domains of functioning, disability and health even in the absence of significant change in dystonia rating scales. (c) 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.ejpn.2011.12.014" target="_blank" rel="noreferrer noopener">10.1016/j.ejpn.2011.12.014</a>
Rights
Information about rights held in and over the resource
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).
(DBS)
2012
BFMDRS
cerebral-palsy
Childhood dystonia
Children
deep brain stimulation
Disability
European Journal of Paediatric Neurology
Follow-up
Gimeno H
globus-pallidus internus
Glutaric acidemia type I
Goals
Lin J P
Neurosciences & Neurology
Outcomes
Paediatric deep brain stimulation
Patient Selection
pediatric movement-disorders
Pediatrics
primary generalized dystonia
Rehabilitation
Reliability
secondary dystonia
Selway R
surgical intervention
tone and motor problems
Tustin K
Validity
-
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
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1002/mds.27129" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/mds.27129</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
A name given to the resource
Clinical rating scale for pantothenate kinase-associated neurodegeneration: A pilot study
Publisher
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Movement Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
Cross-Sectional Studies; Middle Aged; Aged; Young Adult; Child; Humans; Adult; Adolescent; Disabled Persons; Reproducibility of Results; Pilot Projects; Mental Disorders/et [Etiology]; Severity of Illness Index; Dystonia/di [Diagnosis]; Pantothenate Kinase-Associated Neurodegeneration/di [Diagnosis]; Parkinsonian Disorders/di [Diagnosis]; Cognitive Dysfunction/di [Diagnosis]; Cognitive Dysfunction/et [Etiology]; Dystonia/et [Etiology]; Mental Disorders/di [Diagnosis]; Ocular Motility Disorders/di [Diagnosis]; Ocular Motility Disorders/et [Etiology]; Pantothenate Kinase-Associated Neurodegeneration/co [Complications]; Pantothenate Kinase-Associated Neurodegeneration/ge [Genetics]; Parkinsonian Disorders/et [Etiology]; behavior; tone and motor problems; IND; tool development; scale development; PKANDRS;
Creator
An entity primarily responsible for making the resource
Darling A; Tello C; Marti M J; Garrido C; Aguilera-Albesa S; Tomas V M; Gaston I; Madruga M; Gonzalez G L; Ramos L J; Pujol M; Gavilan I T; Tustin K; Lin J P; Zorzi G; Nardocci N; Martorell L; Lorenzo S G; Gutierrez F; Garcia P J; Vela L; Hernandez L C; Ortigoza E J D; Marti S L; Moreira F; Coelho M; Correia G L; Castro C A; Ferreira J; Pires P; Costa C; Rego P; Magalhaes M; Stamelou M; Cuadras P D; Rodriguez-Blazquez C; Martinez-Martin P; Lupo V; Stefanis L; Pons R; Espinos C; Temudo T; Perez D B
Description
An account of the resource
BACKGROUND: Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the assessment of patients with pantothenate kinase-associated neurodegeneration. METHODS: In this international cross-sectional study, patients were examined at the referral centers following a standardized protocol. The motor examination was filmed, allowing 3 independent specialists in movement disorders to analyze 28 patients for interrater reliability assessment. The scale included 34 items (maximal score, 135) encompassing 6 subscales for cognition, behavior, disability, parkinsonism, dystonia, and other neurological signs. RESULTS: Forty-seven genetically confirmed patients (30 +/- 17 years; range, 6-77 years) were examined with the scale (mean score, 62 +/- 21; range, 20-106). Dystonia with prominent cranial involvement and atypical parkinsonian features were present in all patients. Other common signs were cognitive impairment, psychiatric features, and slow and hypometric saccades. Dystonia, parkinsonism, and other neurological features had a moderate to strong correlation with disability. The scale showed good internal consistency for the total scale (Cronbach's alpha = 0.87). On interrater analysis, weighted kappa values (0.30-0.93) showed substantial or excellent agreement in 85% of the items. The scale also discriminated a subgroup of homozygous c.1583C>T patients with lower scores, supporting construct validity for the scale. CONCLUSIONS: The proposed scale seems to be a reliable and valid instrument for the assessment of pediatric and adult patients with pantothenate kinase-associated neurodegeneration. Additional validation studies with a larger sample size will be required to confirm the present results and to complete the scale validation testing. © 2017 International Parkinson and Movement Disorder Society.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/mds.27129" target="_blank" rel="noreferrer noopener">10.1002/mds.27129</a>
Rights
Information about rights held in and over the resource
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).
2017
Adolescent
Adult
Aged
Aguilera-Albesa S
Behavior
Castro C A
Child
Coelho M
Cognitive Dysfunction/di [Diagnosis]
Cognitive Dysfunction/et [Etiology]
Correia G L
Costa C
Cross-sectional Studies
Cuadras P D
Darling A
Disabled Persons
dystonia/di [Diagnosis]
Dystonia/et [Etiology]
Espinos C
Ferreira J
Garcia P J
Garrido C
Gaston I
Gavilan I T
Gonzalez G L
Gutierrez F
Hernandez L C
Humans
IND
Lin J P
Lorenzo S G
Lupo V
Madruga M
Magalhaes M
Marti M J
Marti S L
Martinez-Martin P
Martorell L
Mental Disorders/di [Diagnosis]
Mental Disorders/et [Etiology]
Middle Aged
Moreira F
Movement Disorders
Nardocci N
Ocular Motility Disorders/di [Diagnosis]
Ocular Motility Disorders/et [Etiology]
Ortigoza E J D
Pantothenate Kinase-Associated Neurodegeneration/co [Complications]
Pantothenate Kinase-Associated Neurodegeneration/di [Diagnosis]
Pantothenate Kinase-Associated Neurodegeneration/ge [Genetics]
Parkinsonian Disorders/di [Diagnosis]
Parkinsonian Disorders/et [Etiology]
Perez D B
Pilot Projects
Pires P
PKANDRS
Pons R
Pujol M
Ramos L J
Rego P
Reproducibility of Results
Rodriguez-Blazquez C
scale development
Severity Of Illness Index
Stamelou M
Stefanis L
Tello C
Temudo T
Tomas V M
tone and motor problems
tool development
Tustin K
Vela L
Young Adult
Zorzi G