1 40 1 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 An entity responsible for making the resource available 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