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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
Text
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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
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Clinical rating scale for pantothenate kinase-associated neurodegeneration: A pilot study
Publisher
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Movement Disorders
Date
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2017
Subject
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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
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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
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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
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<a href="http://doi.org/10.1002/mds.27129" target="_blank" rel="noreferrer noopener">10.1002/mds.27129</a>
Rights
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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).
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