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40
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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
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<a href="http://doi.org/10.1007/s00381-017-3361-x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s00381-017-3361-x</a>
Dublin Core
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Title
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Considerations in deep brain stimulation (DBS) for pediatric secondary dystonia
Publisher
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Childs Nervous System
Date
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2017
Subject
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Pediatrics; Surgery; Neurosciences & Neurology; cerebral-palsy; Deep brain stimulation; globus-pallidus internus; hemidystonia; Secondary dystonia; Pediatric neurosurgery; surgical-treatment; tone and motor problems; IND; surgical intervention; deep brain stimulation; dystonia
Creator
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Tsering D; Tochen L; Lavenstein B; Reddy S K; Granader Y; Keating R F; Oluigbo C O
Description
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Purpose There is a paucity of effective long-term medication treatment for secondary dystonias. In situations where significantly impairing secondary dystonias fail to respond to typical enteral medications and intrathecal (or even intraventricular) baclofen, consideration should be given to the use of deep brain stimulation (DBS). While Level I evidence and long-term follow-up clearly demonstrate the efficacy of DBS for primary dystonia, the evidence for secondary dystonia remains mixed and unclear. In this study, we report our experience with pediatric subjects who have undergone DBS for secondary dystonia. Methods We discuss the indications and outcomes of DBS procedures completed at our center. We also present a detailed discussion of the considerations in the management of these patients as well as a literature review. Results Of the four cases retrospectively examined here, all subjects experienced reductions in the severity of their dystonia (ranging from 0 to 100% on both the Barry-Albright Dystonia (BAD) and Burke-Fahn-Marsden Dystonia Rating Scale-Motor (BFMDRS-M) scales). Conclusions Pallidal DBS should be considered among children with functionally debilitating, medication-resistant secondary dystonia. Patients without fixed skeletal deformities who have experienced a short duration of symptoms are most likely to benefit from this intervention.
Identifier
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<a href="http://doi.org/10.1007/s00381-017-3361-x" target="_blank" rel="noreferrer noopener">10.1007/s00381-017-3361-x</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).
2017
cerebral-palsy
Childs Nervous System
deep brain stimulation
Dystonia
globus-pallidus internus
Granader Y
hemidystonia
IND
Keating R F
Lavenstein B
Neurosciences & Neurology
Oluigbo C O
Pediatric neurosurgery
Pediatrics
Reddy S K
secondary dystonia
Surgery
surgical intervention
surgical-treatment
Tochen L
tone and motor problems
Tsering D
-
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/s00415-009-5261-9" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s00415-009-5261-9</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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An evaluation of rating scales utilized for deep brain stimulation for dystonia
Publisher
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Journal of Neurology
Date
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2010
Subject
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Dystonia; Neurosciences & Neurology; Deep brain stimulation; cervical dystonia; globus-pallidus internus; primary generalized dystonia; electrical-stimulation; Globus pallidus internus; movement-disorders; Outcome; pallidotomy; Rating; scales; secondary dystonia; segmental dystonia; term-follow-up; torsion dystonia; tone and motor problems; unspecified Q3 conditions; Q3 conditions; tool development; scale development; surgical intervention; deep brain stimulation
Creator
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Susatia F; Malaty I A; Foote K D; Wu S S; Zeilman P R; Mishra M; Rodriguez R L; ul Haq I; Jacobson C E; Sun A Q; Okun M S
Description
An account of the resource
The objective of this study was to examine globus pallidus internus deep brain stimulation (GPi-DBS) outcomes in primary and secondary dystonia, derived from blinded ratings using two scales and two raters. Twenty-five patients with variable presentations of dystonia were evaluated with videotaped standardized dystonia rating scales at preoperative baseline and at 6 and 12 months following GPi-DBS implantation. These 75 examination videos were retrospectively evaluated, independently and in random order, by two movement disorder neurologists who were blinded to the treatment status. Both neurologists scored each videotaped evaluation using the Burke-Fahn-Marsden Dystonia Rating Scale-motor part (BFMDRS-M) and the Unified Dystonia Rating Scale (UDRS). A final score for each video was assigned by averaging the raters' scores. An intra-class correlation coefficient was used to calculate inter-rater reliability. A linear mixed model was fitted to investigate the time effect and its interaction with type of dystonia (primary versus secondary) for each rating scale. Inter-rater reliability was excellent. Intraclass correlation coefficients ranged from 0.994 to 0.997 for both scales at baseline, 6 and 12 months. The average motor improvement scores after GPi DBS for the entire heterogeneous group of dystonia patients after 6 and 12 months of stimulation was 21.32% (p = 0.0010) and 28.95% (p = 0.0017), respectively, when the UDRS score was used. Similar levels of improvement 20.46% (p = 0.0055) at 6 months and 27.39% (p = 0.00197) at 12 months were found using the BFMDRS-M score. Analysis using unblinded scores from our database revealed a 32.99 and 37.27% UDRS improvement at 6 and 12 months, and an improvement in UDRS score of 38.5 and 43.7% when the analysis was limited to only primary dystonia. If the data were further segregated to include only cases of DYT-1 primary generalized dystonia, the UDRS benefit increased to 48.24%. Our primary dystonia group was diluted by the presence of both old- and young-onset patients, as well as focal, segmental and generalized dystonia. In conclusion, (1) evaluating motor outcomes of DBS therapy for dystonia using independent, randomized retrospective rating by blinded raters' results in lower improvement scores than when outcomes are rated by unblinded treating neurologists. Blinded methodology may be superior and might produce a more realistic assessment of motor outcomes after DBS in patients with dystonia; (2) outcomes were similar whether the BFMDRS-M or UDRS was utilized; (3) GPi-DBS was effective in treating sustained involuntary motor co-contractions in medication refractory dystonia patients, more so in primary dystonia.
Identifier
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<a href="http://doi.org/10.1007/s00415-009-5261-9" target="_blank" rel="noreferrer noopener">10.1007/s00415-009-5261-9</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
cervical dystonia
deep brain stimulation
Dystonia
electrical-stimulation
Foote K D
globus pallidus internus
globus-pallidus internus
Jacobson C E
Journal Of Neurology
Malaty I A
Mishra M
movement-disorders
Neurosciences & Neurology
Okun M S
Outcome
pallidotomy
primary generalized dystonia
Q3 conditions
Rating
Rodriguez R L
scale development
scales
secondary dystonia
segmental dystonia
Sun A Q
surgical intervention
Susatia F
term-follow-up
tone and motor problems
tool development
torsion dystonia
ul Haq I
unspecified Q3 conditions
Wu S S
Zeilman P 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/10.1016/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
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2012
Subject
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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
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Gimeno H; Tustin K; Selway R; Lin J P
Description
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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
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<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/10.3171/2011.8.PEDS11153" target="_blank" rel="noreferrer noopener">http://doi.org/10.3171/2011.8.PEDS11153</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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Deep brain stimulation in children: experience and technical pearls
Publisher
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Journal of Neurosurgery - Pediatrics
Date
A point or period of time associated with an event in the lifecycle of the resource
2011
Subject
The topic of the resource
Adolescent; Adult; Age; Factors Brain/pa [Pathology]; Brain/ra [Radiography]; Child; Preschool; Deep Brain Stimulation/ae [Adverse Effects]; Deep Brain Stimulation/mt [Methods]; Dystonia/pp [Physiopathology]; Dystonia/th [Therapy]; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Movement; Retrospective Studies; Tomography; X-Ray Computed Treatment Outcome; tone and motor problems; Glutaric acidemia type I; Lesch-Nyhan syndrome; deep brain stimulation; secondary dystonia
Creator
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Air E L; Ostrem J L; Sanger T D; Starr P A
Description
An account of the resource
OBJECT: Deep brain stimulation (DBS) is an established technique for the treatment of several movement disorders in adults. However, the technical approach, complications, and results of DBS in children have not been well documented. METHODS: A database of DBS implantations performed at a single institution, prospectively established in 1998, was reviewed for patients who received DBS prior to the age of 18. Diagnoses, surgical technique, and complications were noted. Outcomes were assessed using standard rating scales of neurological function. RESULTS: Of 815 patients undergoing DBS implantation over a 12-year period, 31 were children (mean age at surgery 13.2 years old, range 4-17 years old). Diagnoses included the following: DYT1 primary dystonia (autosomal dominant, Tor1ADELTAGAG mutation, 10 cases), non-DYT1 primary dystonia (3 cases), secondary dystonia (11 cases), neurodegeneration with brain iron accumulation (NBIA, 3 cases), levodopa-responsive parkinsonism (2 cases), Lesch-Nyhan disease (1 case), and glutaric aciduria Type 1 (1 case). Six children ages 15-17 years old underwent awake microelectrode-guided surgery. For 25 children operated under general anesthesia, the surgical technique evolved from microelectrode-guided surgery to image-guided surgeries using real-time intraoperative MR imaging or CT for lead location confirmation. Complications included 5 hardware infections, all in children younger than 10 years old. At 1 year after implantation, patients with DYT1 dystonia had a mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale movement subscore of 75%, while those with secondary dystonia had only small improvements. Outcomes in the 3 children with NBIA were disappointing. CONCLUSIONS: Results of DBS in children with primary and secondary dystonias were similar to those in adults, with excellent results for DYT1 dystonia in children without fixed orthopedic deformity and much more modest results in secondary dystonia. In contrast to reported experience in adults with NBIA, these results in children with NBIA were poor. Infection risk was highest in the youngest patients.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.3171/2011.8.PEDS11153" target="_blank" rel="noreferrer noopener">10.3171/2011.8.PEDS11153</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).
2011
Adolescent
Adult
Age
Air E L
Brain/ra [Radiography]
Child
deep brain stimulation
Deep Brain Stimulation/ae [Adverse Effects]
Deep Brain Stimulation/mt [Methods]
Dystonia/pp [Physiopathology]
Dystonia/th [Therapy]
Factors Brain/pa [Pathology]
Female
Follow-up Studies
Glutaric acidemia type I
Humans
Journal of Neurosurgery - Pediatrics
Lesch-Nyhan syndrome
Magnetic Resonance Imaging
Male
Movement
Ostrem J L
Preschool
Retrospective Studies
Sanger T D
secondary dystonia
Starr P A
Tomography
tone and motor problems
X-Ray Computed Treatment Outcome