1
40
11
-
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/10.1371/journal.pone.0162463" target="_blank" rel="noreferrer noopener">http://doi.org/10.1371/journal.pone.0162463</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
Functional and gait assessment in children and adolescents affected by Friedreich's ataxia: A one-year longitudinal study
Publisher
An entity responsible for making the resource available
PLoS ONE
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
adolescent; reliability; preschool child; longitudinal study; human; article; child; female; male; controlled study; clinical article; walking; Friedreich's ataxia; functional assessment; gait; assessment of humans; Gait Analysis and the Scale for the Assessment and Rating of Ataxia; step length; stride length; walking velocity; tone and motor problems; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Vasco G; Gazzellini S; Petrarca M; Lispi M L; Pisano A; Zazza M; Bella G D; Castelli E; Bertini E
Description
An account of the resource
Friedreich's ataxia is the most common autosomal recessive form of neurodegenerative ataxia. We present a longitudinal study on the gait pattern of children and adolescents affected by Friedreich's ataxia using Gait Analysis and the Scale for the Assessment and Rating of Ataxia (SARA). We assessed the spectrum of changes over 12 months of the gait characteristics and the relationship between clinical and instrumental evaluations. We enrolled 11 genetically confirmed patients affected by Friedreich's ataxia in this study together with 13 normally developing age-matched subjects. Eight patients completed a 12-month follow-up under the same protocol. By comparing the gait parameters of Friedreich's ataxia with the control group, we found significant differences for some relevant indexes. In particular, the increased knee and ankle extension in stance revealed a peculiar biomechanical pattern, which correlated reliably with SARA Total, Gait and Sitting scores. The knee pattern showed its consistency also at the follow-up: Knee extension increased from 6.8+/-3.5degree to -0.5+/-3.7degree and was significantly correlated with the SARA total score. This feature anticipated the loss of the locomotor function in two patients. In conclusion, our findings demonstrate that the selective and segmental analysis of kinetic/kinematic features of ataxic gait, in particular the behavior of the knee, provides sensitive measures to detect specific longitudinal and functional alterations, more than the SARA scale, which however has proved to be a reliable and practical assessment tool. Functional outcomes measures integrated by instrumental evaluation increase their sensitivity, reliability and suitability for the follow-up of the disease progression and for the application in clinical trials and in rehabilitative programs.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1371/journal.pone.0162463" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0162463</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).
2016
Adolescent
Article
assessment of humans
Bella G D
Bertini E
Castelli E
characteristics
Child
Clinical Article
Controlled Study
Female
Friedreich's ataxia
functional assessment
Gait
Gait Analysis and the Scale for the Assessment and Rating of Ataxia
Gazzellini S
Human
Lispi M L
longitudinal study
Male
Petrarca M
Pisano A
PLoS One
Preschool Child
Reliability
step length
stride length
tone and motor problems
Trajectory
Vasco G
Walking
walking velocity
Zazza M
-
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/10.1212/01.WNL.0000156802.15466.79" target="_blank" rel="noreferrer noopener">http://doi.org/10.1212/01.WNL.0000156802.15466.79</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
Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale
Publisher
An entity responsible for making the resource available
Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2005
Subject
The topic of the resource
Adolescent; Adult; Age of Onset; Arm/pp [Physiopathology]; Brain Stem/pp [Physiopathology]; Disease Progression; Female; Friedreich Ataxia/di [Diagnosis]; Friedreich Ataxia/pp [Physiopathology]; Gait Disorders; Neurologic/di [Diagnosis]; Gait Disorders; Neurologic/et [Etiology]; Gait Disorders; tone and motor problems; Friedreich's ataxia; tool development; scale development; Friedrich's Ataxia Scale
Creator
An entity primarily responsible for making the resource
Subramony S H; May W; Lynch D; Gomez C; Fischbeck K; Hallett M; Taylor P; Wilson R; Ashizawa T; Cooperative Ataxia Group
Description
An account of the resource
Measuring the severity of neurologic dysfunction in patients with inherited ataxias, including Friedreich ataxia (FA), is difficult because of the variable rate of progression, the variable age at onset and the variety of neural systems that may be affected. The authors discuss the problems related to rating scales in the ataxias, report a neurologic rating scale for FA, and demonstrate acceptable interrater reliability of the instrument.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1212/01.WNL.0000156802.15466.79" target="_blank" rel="noreferrer noopener">10.1212/01.WNL.0000156802.15466.79</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).
2005
Adolescent
Adult
Age of Onset
Arm/pp [Physiopathology]
Ashizawa T
Brain Stem/pp [Physiopathology]
Cooperative Ataxia Group
Disease Progression
Female
Fischbeck K
Friedreich Ataxia/di [Diagnosis]
Friedreich Ataxia/pp [Physiopathology]
Friedreich's ataxia
Friedrich's Ataxia Scale
Gait Disorders
Gomez C
Hallett M
Lynch D
May W
Neurologic/di [Diagnosis]
Neurologic/et [Etiology]
Neurology
scale development
Subramony S H
Taylor P
tone and motor problems
tool development
Wilson R
-
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/10.1186/s13023-017-0709-y" target="_blank" rel="noreferrer noopener">http://doi.org/10.1186/s13023-017-0709-y</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
Urinary, bowel and sexual symptoms in a cohort of patients with Friedreich's ataxia
Publisher
An entity responsible for making the resource available
Orphanet Journal of Rare Diseases
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
bowel incontinence; urinary incontinence; Friedreich's ataxia; trajectory; characteristics; lower UTI symptoms; bowel symptoms
Creator
An entity primarily responsible for making the resource
Lad M; Parkinson M H; Rai M; Pandolfo M; Bogdanova-Mihaylova P; Walsh R A; Murphy S; Emmanuel A; Panicker J; Giunti P
Description
An account of the resource
Background: Pelvic symptoms are distressing symptoms experienced by patients with Friedreich's Ataxia (FRDA). The aim of this study was to describe the prevalence of lower urinary tract symptoms (LUTS), bowel and sexual symptoms in FRDA.Methods: Questionnaire scores measuring LUTS, bowel and sexual symptoms were analysed with descriptive statistics as a cohort and as subgroups (Early/Late-onset and Early/Late-stage FRDA) They were also correlated with validated measures of disease severity including those of ataxia severity, non-ataxic symptoms and activities of daily living.Results:
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1186/s13023-017-0709-y" target="_blank" rel="noreferrer noopener">10.1186/s13023-017-0709-y</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
Bogdanova-Mihaylova P
bowel incontinence
bowel symptoms
characteristics
Emmanuel A
Friedreich's ataxia
Giunti P
Lad M
lower UTI symptoms
Murphy S
Orphanet Journal Of Rare Diseases
Pandolfo M
Panicker J
Parkinson M H
Rai M
Trajectory
urinary incontinence
Walsh R 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
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/10.1007/s00455-017-9804-4" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s00455-017-9804-4</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
Dysphagia in Friedreich Ataxia
Publisher
An entity responsible for making the resource available
Dysphagia
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
Quality of life; speech; management; Otorhinolaryngology; Swallowing; adults; Gait disorders/ataxia; interrater reliability; molecular-genetics; pathogenesis; pathology; penetration-aspiration scale; Trinucleotide repeat diseases; Videofluoroscopy; feeding difficulties; Friedreich's ataxia; trajectory; characteristics; dysphagia; penetration; aspiration
Creator
An entity primarily responsible for making the resource
Keage M J; Delatycki M B; Gupta I;Corben L A; Vogel A P
Description
An account of the resource
The objective of the study was to comprehensively characterise dysphagia in Friedreich ataxia (FRDA) and identify predictors of penetration/aspiration during swallowing. We also investigated the psychosocial impact of dysphagia on individuals with FRDA. Sixty participants with FRDA were screened for dysphagia using a swallowing quality of life questionnaire (Swal-QOL) and case history. Individuals reporting dysphagia underwent a standardised oromotor assessment (Frenchay Dysarthria Assessment, 2, FDA-2) and videofluoroscopic study of swallowing (VFSS). Data were correlated with disease parameters (age at symptom onset, age at assessment, disease duration, FXN intron 1 GAA repeat sizes, and Friedreich Ataxia Rating Scale (FARS) score). Predictors of airway penetration/aspiration were explored using logistic regression analysis. Ninety-eight percent (59/60) of participants reported dysphagia, of whom 35 (58.3%) underwent FDA-2 assessment, and 38 (63.3%) underwent VFSS. Laryngeal, respiratory, and tongue dysfunction was observed on the FDA-2. A Penetration-Aspiration Scale score above 3 (deemed significant airway compromise based on non-clinical groups) was observed on at least one consistency in 13/38 (34.2%) participants. All of those who aspirated (10/38, 26.3%) did so silently, with no overt signs of airway entry such as reflexive cough. Significant correlations were observed between dysphagic symptoms and disease duration and severity. No reliable predictors of penetration or aspiration were identified. Oropharyngeal dysphagia is commonly present in individuals with FRDA and worsens with disease duration and severity. Individuals with FRDA are at risk of aspiration at any stage of the disease and should be reviewed regularly. Instrumental analysis remains the only reliable method to detect aspiration in this population. Dysphagia significantly affects the quality of life of individuals with FRDA.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/s00455-017-9804-4" target="_blank" rel="noreferrer noopener">10.1007/s00455-017-9804-4</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
adults
aspiration
characteristics
Corben L A
Delatycki M B
Dysphagia
feeding difficulties
Friedreich's ataxia
Gait disorders/ataxia
Gupta I
interrater reliability
Keage M J
Management
molecular-genetics
Otorhinolaryngology
pathogenesis
Pathology
penetration
penetration-aspiration scale
Quality Of Life
Speech
Swallowing
Trajectory
Trinucleotide repeat diseases
Videofluoroscopy
Vogel A P
-
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/10.1212/WNL.0b013e3182749e67" target="_blank" rel="noreferrer noopener">http://doi.org/10.1212/WNL.0b013e3182749e67</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
Video game-based coordinative training improves ataxia in children with degenerative ataxia
Publisher
An entity responsible for making the resource available
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
tone and motor problems; Friedreich's ataxia; physical intervention; video game-based coordinative training; ataxia; balance
Creator
An entity primarily responsible for making the resource
Ilg W; Schatton C; Schicks J; Giese M A; Schols L; Synofzik M
Description
An account of the resource
Objective: Degenerative ataxias in children present a rare condition where effective treatments are lacking. Intensive coordinative training based on physiotherapeutic exercises improves degenerative ataxia in adults, but such exercises have drawbacks for children, often including a lack of motivation for high-frequent physiotherapy. Recently developed whole-body controlled video game technology might present a novel treatment strategy for highly interactive and motivational coordinative training for children with degenerative ataxias. Methods: We examined the effectiveness of an 8-week coordinative training for 10 children with progressive spinocerebellar ataxia. Training was based on 3 Microsoft Xbox Kinect video games particularly suitable to exercise whole-body coordination and dynamic balance. Training was started with a laboratory-based 2-week training phase and followed by 6 weeks training in children's home environment. Rater-blinded assessments were performed 2 weeks before laboratorybased training, immediately prior to and after the laboratory-based training period, as well as after home training. These assessments allowed for an intraindividual control design, where performance changes with and without training were compared. Results: Ataxia symptoms were significantly reduced (decrease in Scale for the Assessment and Rating of Ataxia score, p = 0.0078) and balance capacities improved (dynamic gait index, p = 0.04) after intervention. Quantitative movement analysis revealed improvements in gait (lateral sway: p = 0.01; step length variability: p = 0.01) and in goal-directed leg placement (p 5 0.03). Conclusions: Despite progressive cerebellar degeneration, children are able to improvemotor performance by intensive coordination training. Directed training of whole-body controlled video games might present a highly motivational, cost-efficient, and home-based rehabilitation strategy to train dynamic balance and interaction with dynamic environments in a large variety of youngonset neurologic conditions. Classification of evidence: This study provides Class III evidence that directed training with Xbox Kinect video games can improve several signs of ataxia in adolescents with progressive ataxia as measured by SARA score, Dynamic Gait Index, and Activity-specific Balance Confidence Scale at 8 weeks of training. © 2012 American Academy of Neurology.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1212/WNL.0b013e3182749e67" target="_blank" rel="noreferrer noopener">10.1212/WNL.0b013e3182749e67</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).
2012
ataxia
balance
Friedreich's ataxia
Giese M A
Ilg W
Neurology
physical intervention
Schatton C
Schicks J
Schols L
Synofzik M
tone and motor problems
video game-based coordinative training
-
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/10.1186/s12984-015-0032-6" target="_blank" rel="noreferrer noopener">http://doi.org/10.1186/s12984-015-0032-6</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
Robotic and clinical evaluation of upper limb motor performance in patients with Friedreich's Ataxia: an observational study
Publisher
An entity responsible for making the resource available
Journal of Neuroengineering and Rehabilitation
Date
A point or period of time associated with an event in the lifecycle of the resource
2015
Subject
The topic of the resource
children; Rehabilitation; Neurosciences & Neurology; Engineering; coordination; therapy; rating-scale; Accuracy; and Rating of Ataxia; cerebellar-ataxia; Friedreich's ataxia; Kinematics; movement; multiple-sclerosis; quantitative-evaluation; Reaching task; rehabilitation; Robot-mediated evaluation; Scale for the Assessment; smoothness; Smoothness; stroke recovery; Submovements; Upper limb; tone and motor problems; tool development; scale development; InMotion Arm Robot
Creator
An entity primarily responsible for making the resource
Germanotta M; Vasco G; Petrarca M; Rossi S; Carniel S; Bertini E; Cappa P; Castelli E
Description
An account of the resource
Background: Friedreich's ataxia (FRDA) is the most common hereditary autosomal recessive form of ataxia. In this disease there is early manifestation of gait ataxia, and dysmetria of the arms and legs which causes impairment in daily activities that require fine manual dexterity. To date there is no cure for this disease. Some novel therapeutic approaches are ongoing in different steps of clinical trial. Development of sensitive outcome measures is crucial to prove therapeutic effectiveness. The aim of the study was to assess the reliability and sensitivity of quantitative and objective assessment of upper limb performance computed by means of the robotic device and to evaluate the correlation with clinical and functional markers of the disease severity. Methods: Here we assess upper limb performances by means of the InMotion Arm Robot, a robot designed for clinical neurological applications, in a cohort of 14 children and young adults affected by FRDA, matched for age and gender with 18 healthy subjects. We focused on the analysis of kinematics, accuracy, smoothness, and submovements of the upper limb while reaching movements were performed. The robotic evaluation of upper limb performance consisted of planar reaching movements performed with the robotic system. The motors of the robot were turned off, so that the device worked as a measurement tool. The status of the disease was scored using the Scale for the Assessment and Rating of Ataxia (SARA). Relationships between robotic indices and a range of clinical and disease characteristics were examined. Results: All our robotic indices were significantly different between the two cohorts except for two, and were highly and reliably discriminative between healthy and subjects with FRDA. In particular, subjects with FRDA exhibited slower movements as well as loss of accuracy and smoothness, which are typical of the disease. Duration of Movement, Normalized Jerk, and Number of Submovements were the best discriminative indices, as they were directly and easily measurable and correlated with the status of the disease, as measured by SARA. Conclusions: Our results suggest that outcome measures obtained by means of robotic devices can improve the sensitivity of clinical evaluations of patients' dexterity and can accurately and efficiently quantify changes over time in clinical trials, particularly when functional scales appear to be no longer sensitive.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1186/s12984-015-0032-6" target="_blank" rel="noreferrer noopener">10.1186/s12984-015-0032-6</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).
2015
accuracy
and Rating of Ataxia
Bertini E
Cappa P
Carniel S
Castelli E
cerebellar-ataxia
Children
Coordination
Engineering
Friedreich's ataxia
Germanotta M
InMotion Arm Robot
Journal of Neuroengineering and Rehabilitation
Kinematics
Movement
multiple-sclerosis
Neurosciences & Neurology
Petrarca M
quantitative-evaluation
rating-scale
Reaching task
Rehabilitation
Robot-mediated evaluation
Rossi S
scale development
Scale for the Assessment
smoothness
stroke recovery
Submovements
Therapy
tone and motor problems
tool development
Upper limb
Vasco G
-
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/10.1097/NPT.0b013e3181b5112e" target="_blank" rel="noreferrer noopener">http://doi.org/10.1097/NPT.0b013e3181b5112e</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
Characterizing gait, locomotor status, and disease severity in children and adolescents with Friedreich ataxia
Publisher
An entity responsible for making the resource available
Journal of Neurologic Physical Therapy
Date
A point or period of time associated with an event in the lifecycle of the resource
2009
Subject
The topic of the resource
tone and motor problems; Friedreich's ataxia; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Croarkin E; Maring J; Pfalzer L; Harris-Love M; Siegel K; DiProspero N
Description
An account of the resource
BACKGROUND AND PURPOSE: The purpose of this study was to describe gait parameters in children and adolescents with a diagnosis of Friedreich ataxia (FA) and examine the relationship between disease severity, measured by the Friedreich Ataxia Rating Scale (FARS) and gait parameters. The study examined whether FARS scores can discriminate between those who walk independently and those who require assistance. METHODS: Thirty-eight children (aged 5-11 years) and adolescents (aged 12-17 years) with genetically confirmed FA were divided into two groups based on locomotor status: group 1, subjects who were able to walk independently, and group 2, subjects who required assistance for walking. Temporal and spatial gait parameters were collected using the Stride Analyzer computerized foot switch system and compared with age-matched normative data. The FARS was used to measure disease severity. Correlation coefficients and the Mann-Whitney U test of differences were used to evaluate associations and discern differences between groups. RESULTS: In subjects with FA, gait parameters of velocity and cadence were slower and stride length was shorter compared with age-matched children without disabilities. These parameters were significantly correlated with FARS score (r = 0.696, 0.667, 0.537; respectively, all P values <0.001). Total FARS scores were correlated with locomotor status (c value r = 0.623; P < 0.01) and could categorize subjects into groups based on independent walking or need for assistance, 73% and 87% of the time, respectively. DISCUSSION AND CONCLUSION: Subjects with FA exhibited specific abnormal gait characteristics relative to age-matched individuals. Disease severity, as measured by the FARS, was associated with gait velocity, stride length, and cadence. FARS scores can be used to categorize subjects by locomotor status and may be a useful screening tool to identify those requiring assistance.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/NPT.0b013e3181b5112e" target="_blank" rel="noreferrer noopener">10.1097/NPT.0b013e3181b5112e</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).
2009
characteristics
Croarkin E
DiProspero N
Friedreich's ataxia
Harris-Love M
Journal of Neurologic Physical Therapy
Maring J
Pfalzer L
Siegel K
tone and motor problems
Trajectory
-
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/10.1007/s00415-009-5352-7" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s00415-009-5352-7</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
A comparison of three measures of upper limb function in Friedreich ataxia
Publisher
An entity responsible for making the resource available
Journal of Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2010
Subject
The topic of the resource
tone and motor problems; Friedreich's ataxia; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Corben L A; Tai G; Wilson C; Collins V; Churchyard A J; Delatycki M B
Description
An account of the resource
Friedreich Ataxia (FRDA) is the commonest inherited ataxia. Clinical trials of pharmaceuticals are increasingly being conducted in this condition. This requires the most accurate outcome measures to enable trials to be conducted with a minimum number of subjects in the shortest time frame and to minimize the risk of false negative results. Upper limb function is a major area of morbidity in FRDA. We therefore have compared the performance of three tests of upper limb function in FRDA: the Nine Hole Peg Test (9HPT), Box and Blocks Test (BBT) and Jebsen Taylor Hand Function Test (JTHFT). This study was undertaken to ascertain the best test for inclusion in a Friedreich Ataxia Functional Composite (FAFC) test for use in clinical studies and therapeutic trials. The three tests were administered to the dominant and non-dominant upper limbs of 38 individuals with genetically proven FRDA on two occasions, 12 months apart. The results of testing were correlated with the following disease parameters; age at disease onset, disease duration and score for the Friedreich Ataxia Rating Scale (FARS). The responsiveness to change of each test was assessed by measuring the effect size and calculations of the number of subjects required for similarly powered therapeutic trials. Results for all tests correlated significantly with disease duration and FARS score. The only test scores that changed significantly over 12 months were those for the non-dominant 9HPT and BBT. Scores for these two tests also had the largest effect sizes and required the fewest subjects for similarly powered therapeutic trials. We conclude, therefore, that the non-dominant 9HPT and BBT are the best tests for inclusion in a FAFC. Since the 9HPT has already been suggested for inclusion in a FAFC, we recommend that this test is used but that it is the non-dominant limb that is tested.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/s00415-009-5352-7" target="_blank" rel="noreferrer noopener">10.1007/s00415-009-5352-7</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).
2010
characteristics
Churchyard A J
Collins V
Corben L A
Delatycki M B
Friedreich's ataxia
Journal Of Neurology
Tai G
tone and motor problems
Trajectory
Wilson C
-
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/10.1212/WNL.0b013e318297ef18" target="_blank" rel="noreferrer noopener">http://doi.org/10.1212/WNL.0b013e318297ef18</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
Increased prevalence of sleep-disordered breathing in Friedreich ataxia
Publisher
An entity responsible for making the resource available
Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2013
Subject
The topic of the resource
reliability; Neurosciences & Neurology; disease; scale; adults; apnea syndrome; daytime sleepiness; breathing difficulties; Friedreich's ataxia; trajectory; characteristics; obstructive sleep apnea
Creator
An entity primarily responsible for making the resource
Corben L A; Ho M; Copland J; Tai G; Delatycki M B
Description
An account of the resource
Objectives: We sought to document the prevalence and nature of sleep-disordered breathing (SDB) in individuals with Friedreich ataxia (FRDA) as well as establish the relationship, if any, between SDB and clinical parameters of FRDA. Methods: Eighty-two individuals with FRDA were administered the Epworth Sleepiness Scale on an annual basis for up to 3 years. Individuals were referred for a sleep study if they had an Epworth Sleepiness Scale score >8 or had clinical symptoms suggestive of SDB. Results: From this cohort, 21 individuals underwent a sleep study and 17 were diagnosed with obstructive sleep apnea syndrome, giving a minimum prevalence of 21%, which is greater than that found in the general population (3%-7%). Moreover, the presence of obstructive sleep apnea was significantly correlated with the duration of disease and clinical severity of FRDA. Conclusion: It is recommended that individuals with FRDA undergo regular screening for obstructive sleep apnea to identify the need for a sleep study and subsequent treatment if SDB is diagnosed.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1212/WNL.0b013e318297ef18" target="_blank" rel="noreferrer noopener">10.1212/WNL.0b013e318297ef18</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).
2013
adults
apnea syndrome
breathing difficulties
characteristics
Copland J
Corben L A
daytime sleepiness
Delatycki M B
Disease
Friedreich's ataxia
Ho M
Neurology
Neurosciences & Neurology
obstructive sleep apnea
Reliability
Scale
Tai G
Trajectory
-
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.20651" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/mds.20651</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
International Cooperative Ataxia Rating Scale (ICARS): Appropriate for studies of Friedreich's ataxia?
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
2005
Subject
The topic of the resource
tone and motor problems; friedreich's ataxia; tool development; scale development
Creator
An entity primarily responsible for making the resource
Cano S J; Hobart J C; Hart P E; Korlipara L V P; Schapira A H V; Cooper J M
Description
An account of the resource
Clinicians require scientifically rigorous, clinically meaningful rating scales to evaluate the health impact of disease and treatment that cannot be measured using conventional laboratory instruments. This study evaluated the psychometric properties of the International Cooperative Ataxia Rating Scale (ICARS), a commonly used clinician-rated measure, in Friedreich's ataxia (FRDA). People with confirmed FRDA were assessed by using the ICARS. Two assumptions of its measurement model were tested: the legitimacy of reporting IC-ARS scores in FRDA, and the acceptability, reliability, and validity of total and subscale scores. Seventy-seven people with FRDA were assessed. The ICARS total score effectively satisfied all psychometric criteria tested. The posture and gait disturbances subscale also performed well. The other three subscales did not pass standard criteria for tests of scaling assumptions, reliability, and validity. This small study recommends only the use of the ICARS total score as a measure of FRDA. However, the extent to which this score quantifies the true extent of FRDA remains uncertain as our validity testing was limited, partly by the lack of appropriate validating measures. Further validity testing, and examination of responsiveness, is required before the ICARS can be recommended as an outcome measure for treatment trials of FDRA. © 2005 Movement Disorder Society.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/mds.20651" target="_blank" rel="noreferrer noopener">10.1002/mds.20651</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).
2005
Cano S J
Cooper J M
Friedreich's ataxia
Hart P E
Hobart J C
Korlipara L V P
Movement Disorders
scale development
Schapira A H V
tone and motor problems
tool development
-
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.22660" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/mds.22660</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
Comparison of three clinical rating scales in Friedreich ataxia (FRDA)
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
2009
Subject
The topic of the resource
tone and motor problems; Friedreich's ataxia; trajectory; characteristics; ataxia
Creator
An entity primarily responsible for making the resource
Bürk K; Mälzig U; Wolf S; Heck S; Dimitriadis K; Schmitz-Hübsch T; Hering S; Lindig T M; Haug V; Timmann D; Degen I; Kruse B; Dörr J M; Ratzka S; Ivo A; Schols L; Boesch S; Klockgether T; Klopstock T; Schulz J B
Description
An account of the resource
To test the validity and reliability of the scale for the assessment and rating of ataxia (SARA) in Friedreich ataxia (FRDA). SARA is limited to eight items and can be performed rapidly. Ninety-six patients with a molecular genetic diagnosis of FRDA were rated using three different clinical scales, the FRDA Rating Scale (FARS), the International Cooperative Ataxia Rating Scale (ICARS), and SARA. Despite considerable discrepancies in scale size and subscale structure, SARA total scores were significantly correlated with ICARS (r = 0.953, P < 0.0001) and FARS (r = 0.938, P < 0.0001) total scores. SARA total scores also correlated with the activities of daily living (ADL, r = 0.929, P < 0.0001). Although originally developed for the use in dominantly inherited ataxias, which are primarily ataxias of the cerebellar type, SARA can also be used successfully to assess afferent ataxia, which is the predominant form in FRDA. Because SARA is characterized by high interrater reliability and practicability, SARA is applicable and well suited forclinical trials of FRDA.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/mds.22660" target="_blank" rel="noreferrer noopener">10.1002/mds.22660</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).
2009
ataxia
Boesch S
Bürk K
characteristics
Degen I
Dimitriadis K
Dörr J M
Friedreich's ataxia
Haug V
Heck S
Hering S
Ivo A
Klockgether T
Klopstock T
Kruse B
Lindig T M
Mälzig U
Movement Disorders
Ratzka S
Schmitz-Hübsch T
Schols L
Schulz J B
Timmann D
tone and motor problems
Trajectory
Wolf S