1
40
10
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Title
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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
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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>
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Title
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Considerations in deep brain stimulation (DBS) for pediatric secondary dystonia
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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
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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/10.1093/brain/awq022" target="_blank" rel="noreferrer noopener">http://doi.org/10.1093/brain/awq022</a>
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Title
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Dystonia in neurodegeneration with brain iron accumulation: outcome of bilateral pallidal stimulation
Publisher
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Brain
Date
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2010
Subject
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Male; Treatment Outcome; Young Adult; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Retrospective Studies; Brain Diseases/physiopathology/therapy; Brain/physiopathology; Deep Brain Stimulation/adverse effects/methods; Dystonia/physiopathology/therapy; Functional Laterality; Globus Pallidus/physiopathology; Iron/metabolism; Neurodegenerative Diseases/physiopathology/therapy; tone and motor problems; IND; surgical intervention; bilateral pallidal stimulation; dystonia
Creator
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Timmermann L; Pauls K A; Wieland K; Jech R; Kurlemann G; Sharma N; Gill S S; Haenggeli C A; Hayflick S J; Hogarth P; Leenders K L; Limousin P; Malanga C J; Moro E; Ostrem J L; Revilla F J; Santens P; Schnitzler A; Tisch S; Valldeoriola F; Vesper J; Volkmann J; Woitalla D; Peker S
Description
An account of the resource
Neurodegeneration with brain iron accumulation encompasses a heterogeneous group of rare neurodegenerative disorders that are characterized by iron accumulation in the brain. Severe generalized dystonia is frequently a prominent symptom and can be very disabling, causing gait impairment, difficulty with speech and swallowing, pain and respiratory distress. Several case reports and one case series have been published concerning therapeutic outcome of pallidal deep brain stimulation in dystonia caused by neurodegeneration with brain iron degeneration, reporting mostly favourable outcomes. However, with case studies, there may be a reporting bias towards favourable outcome. Thus, we undertook this multi-centre retrospective study to gather worldwide experiences with bilateral pallidal deep brain stimulation in patients with neurodegeneration with brain iron accumulation. A total of 16 centres contributed 23 patients with confirmed neurodegeneration with brain iron accumulation and bilateral pallidal deep brain stimulation. Patient details including gender, age at onset, age at operation, genetic status, magnetic resonance imaging status, history and clinical findings were requested. Data on severity of dystonia (Burke Fahn Marsden Dystonia Rating Scale-Motor Scale, Barry Albright Dystonia Scale), disability (Burke Fahn Marsden Dystonia Rating Scale-Disability Scale), quality of life (subjective global rating from 1 to 10 obtained retrospectively from patient and caregiver) as well as data on supportive therapy, concurrent pharmacotherapy, stimulation settings, adverse events and side effects were collected. Data were collected once preoperatively and at 2-6 and 9-15 months postoperatively. The primary outcome measure was change in severity of dystonia. The mean improvement in severity of dystonia was 28.5% at 2-6 months and 25.7% at 9-15 months. At 9-15 months postoperatively, 66.7% of patients showed an improvement of 20% or more in severity of dystonia, and 31.3% showed an improvement of 20% or more in disability. Global quality of life ratings showed a median improvement of 83.3% at 9-15 months. Severity of dystonia preoperatively and disease duration predicted improvement in severity of dystonia at 2-6 months; this failed to reach significance at 9-15 months. The study confirms that dystonia in neurodegeneration with brain iron accumulation improves with bilateral pallidal deep brain stimulation, although this improvement is not as great as the benefit reported in patients with primary generalized dystonias or some other secondary dystonias. The patients with more severe dystonia seem to benefit more. A well-controlled, multi-centre prospective study is necessary to enable evidence-based therapeutic decisions and better predict therapeutic outcomes.
Identifier
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<a href="http://doi.org/10.1093/brain/awq022" target="_blank" rel="noreferrer noopener">10.1093/brain/awq022</a>
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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
Adolescent
Adult
bilateral pallidal stimulation
Brain
Brain Diseases/physiopathology/therapy
Brain/physiopathology
Child
Child Preschool
Deep Brain Stimulation/adverse effects/methods
Dystonia
Dystonia/physiopathology/therapy
Female
Functional Laterality
Gill S S
Globus Pallidus/physiopathology
Haenggeli C A
Hayflick S J
Hogarth P
Humans
IND
Infant
Iron/metabolism
Jech R
Kurlemann G
Leenders K L
Limousin P
Malanga C J
Male
Moro E
Neurodegenerative Diseases/physiopathology/therapy
Ostrem J L
Pauls K A
Peker S
Retrospective Studies
Revilla F J
Santens P
Schnitzler A
Sharma N
surgical intervention
Timmermann L
Tisch S
tone and motor problems
Treatment Outcome
Valldeoriola F
Vesper J
Volkmann J
Wieland K
Woitalla D
Young Adult
-
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/10.1371/journal.pone.0146644" target="_blank" rel="noreferrer noopener">http://doi.org/10.1371/journal.pone.0146644</a>
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Title
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Long-term clinical outcome of internal globus pallidus deep brain stimulation for dystonia
Publisher
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PLoS ONE
Date
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2016
Subject
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tone and motor problems; IND; surgical intervention; deep brain stimulation
Creator
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Park H R; Lee J M; Ehm G; Yang H J; Song I H; Lim Y H; Kim M R; Kim K R; Lee W W; Kim Y E; Hwang J H; Shin C W; Park H; Kim J W; Kim H J; Kim C; Kim D G; Jeon B S; Paek S H
Description
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Background: GPi (Internal globus pallidus) DBS (deep brain stimulation) is recognized as a safe, reliable, reversible and adjustable treatment in patients with medically refractory dystonia. Objectives: This report describes the long-term clinical outcome of 36 patients implanted with GPi DBS at the Neurosurgery Department of Seoul National University Hospital. Methods: Nine patients with a known genetic cause, 12 patients with acquired dystonia, and 15 patients with isolated dystonia without a known genetic cause were included. When categorized by phenomenology, 29 patients had generalized, 5 patients had segmental, and 2 patients had multifocal dystonia. Patients were assessed preoperatively and at defined follow-up examinations postoperatively, using the Burke-Fahn-Marsden dystonia rating scale (BFMDRS) for movement and functional disability assessment. The mean follow-up duration was 47 months (range, 12-84) Results: The mean movement scores significantly decreased from 44.88 points preoperatively to 26.45 points at 60-month follow up (N = 19, P = 0.006). The mean disability score was also decreased over time, from 11.54 points preoperatively to 8.26 points at 60-month follow up, despite no statistical significance (N = 19, P = 0.073). When analyzed the movement and disability improvement rates at 12-month follow up point, no significant difference was noted according to etiology, disease duration, age at surgery, age of onset, and phenomenology. However, the patients with DYT-1 dystonia and isolated dystonia without a known genetic cause showed marked improvement. Conclusions: GPi DBS is a safe and efficient therapeutic method for treatment of dystonia patients to improve both movement and disability. However, this study has some limitations caused by the retrospective design with small sample size in a single-center.
Identifier
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<a href="http://doi.org/10.1371/journal.pone.0146644" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0146644</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).
2016
deep brain stimulation
Ehm G
Hwang J H
IND
Jeon B S
Kim C
Kim D G
Kim H J
Kim J W
Kim K R
Kim M R
Kim Y E
Lee J M
Lee W W
Lim Y H
Paek S H
Park H
Park H R
PLoS One
Shin C W
Song I H
surgical intervention
tone and motor problems
Yang H J
-
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/10.1111/ner.12549" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/ner.12549</a>
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Title
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Subthalamic Nuclei Stimulation in Patients With Pantothenate Kinase-Associated Neurodegeneration (PKAN)
Publisher
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Neuromodulation
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
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adolescent; subthalamic nucleus; follow up; scoring system; human; article; female; male; adult; middle aged; dystonia; case report; treatment outcome; brain depth stimulation; Deep brain stimulation; neurologic disease assessment; Burke Fahn Marsden Dystonia Rating Scale movement rating scale; fluency disorder; globus pallidus internus; neurodegeneration with brain iron accumulation/th [Therapy]; neurologic examination; pantothenate kinase-associated neurodegeneration; subthalamic nuclei; tone and motor problems; IND; surgical intervention; subthalamic nuclei stimulation
Creator
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Liu Z; Liu Y; Yang Y; Wang L; Dou W; Guo J; Wang Y; Guo Y; Wan X; Ma W; Wang R
Description
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Introduction: Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as dystonia, ataxia, dysarthria, and involuntary movements. Treatment of PKAN with deep brain stimulation (DBS) has been reported, but mainly focuses on targeting the globus pallidus internus (GPi). Subthalamic nuclei (STN) may also be a potential target for treatment of PKAN. Methods: In this study, we reviewed three patients with PKAN (two with typical PKAN and one with atypical PKAN) treated by bilateral STN stimulation and present a review of the literature. All patients received neurological evaluation using the Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFMDRS) scoring system before and after surgery. Patients were then subject to regular clinical follow-ups (ranging from 22 to 44 months). Results: The mean stimulation amplitude, pulse width and frequency was 2.65 +/- 0.45 V, 91.7 +/- 21.9 mus, and 146.7 +/- 12.5 Hz, respectively. BFMDRS scores were improved in all patients after surgery, ranging from 41.6 to 73.1%. Improvements of appendicular symptoms ranged from 46.2 to 94.1%, and improvements of axial symptoms ranged from 27.3 to 33.3%. No side effects were reported in patients 1 and 2; whereas patient 3 exhibited a mild decline in verbal fluency one year after surgery. Conclusion: STN stimulation could serve as a candidate DBS target in the treatment of PKAN, especially for patients with prominent appendicular symptoms. Copyright © 2017 International Neuromodulation Society
Identifier
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<a href="http://doi.org/10.1111/ner.12549" target="_blank" rel="noreferrer noopener">10.1111/ner.12549</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
Adolescent
Adult
Article
Brain Depth Stimulation
Burke Fahn Marsden Dystonia Rating Scale movement rating scale
Case Report
deep brain stimulation
Dou W
Dystonia
Female
fluency disorder
Follow Up
globus pallidus internus
Guo J
Guo Y
Human
IND
Liu Y
Liu Z
Ma W
Male
Middle Aged
neurodegeneration with brain iron accumulation/th [Therapy]
neurologic disease assessment
Neurologic Examination
Neuromodulation
pantothenate kinase-associated neurodegeneration
scoring system
subthalamic nuclei
subthalamic nuclei stimulation
Subthalamic Nucleus
surgical intervention
tone and motor problems
Treatment Outcome
Wan X
Wang L
Wang R
Wang Y
Yang Y
-
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.2015.09.007" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ejpn.2015.09.007</a>
Dublin Core
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Title
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Gabapentin can significantly improve dystonia severity and quality of life in children
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
2016
Subject
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tone and motor problems; IND; pharmacologic intervention; gabapentin; involuntary muscle contractions
Creator
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Liow N Y; Gimeno H; Lumsden D E; Marianczak J; Kaminska M; Tomlin S; Lin J S
Description
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INTRODUCTION: Gabapentin has been used in the management of neuropathic pain, epilepsy and occasionally movement disorders. METHODS: A four-year retrospective, observational study analysed the use of gabapentin for severe dystonia in children at the Evelina London Children's Hospital. Motor severity was classified according to the Gross Motor Function Classification System (GMFCS), Dystonia Severity Assessment Plan (DSAP) and levels of impairment in activities of daily living were graded according to the WHO International Classification of Functioning, Disability and Health, Children & Youth version (ICF-CY) before and after gabapentin. RESULTS: The majority of the 69 children reported were level 5 GMFCS (non-ambulant). The DSAP grade fell significantly from grade 3 before to grade 1 after gabapentin. Significant improvements in median ICF-CY grades were seen following gabapentin in sleep quality, sleep amount, mood & agreeableness, pain, general muscle tone, involuntary muscle contractions and seating tolerance (p < 0.01 in all areas). A significantly higher mean dose of 18.1 mg/kg/dose (SD: 13.3) for dystonia, compared to 7.61 mg/kg/dose (SD: 4.14) for pain relief without dystonia (z = -2.54, p = 0.011) was noted. DISCUSSION & CONCLUSION: Gabapentin may significantly ameliorate dystonia severity and improve activities of daily living and quality of life in children with severe dystonia.
Identifier
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<a href="http://doi.org/10.1016/j.ejpn.2015.09.007" target="_blank" rel="noreferrer noopener">10.1016/j.ejpn.2015.09.007</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).
2016
European Journal of Paediatric Neurology
Gabapentin
Gimeno H
IND
involuntary muscle contractions
Kaminska M
Lin J S
Liow N Y
Lumsden D E
Marianczak J
pharmacologic intervention
Tomlin S
tone and motor problems
-
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/10.1111/j.1468-1331.2011.03589.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1468-1331.2011.03589.x</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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Pantothenate kinase-associated neurodegeneration in Korea: recurrent R440P mutation in PANK2 and outcome of deep brain stimulation
Publisher
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European Journal of Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2012
Subject
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tone and motor problems; IND; surgical intervention; Pallidal deep brain stimulation
Creator
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Lim B C; Ki C S; Cho A; Hwang H; Kim K J; Hwang Y S; Kim Y E; Yun J Y; Jeon B S; Lim Y H; Paek S H; Chae J H
Description
An account of the resource
BACKGROUND AND PURPOSE: The purpose of this study was to evaluate the mutation status of PANK2 among Korean patients with pantothenate kinase-associated neurodegeneration (PKAN) and to document the outcome of pallidal deep brain stimulation (DBS). METHODS: Direct sequencing and deletion/duplication analysis of PANK2 were conducted in 12 patients (11 unrelated) with PKAN, diagnosed on the basis of extrapyramidal dysfunction and the 'eye-of-the-tiger sign' on brain magnetic resonance imaging (MRI). Pallidal DBS was conducted in four patients, and the outcomes were measured using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). RESULTS: A PANK2 mutation was identified in both alleles in all patients. The most prevalent mutation was c.1319G>C (p.R440P) in 8/22 mutated alleles (36%). An intragenic deletion ranging from exons 2 to 4 was found in one allele (1/22, 4.5%) using deletion/duplication analysis. The outcome of pallidal DBS was favorable in two patients with atypical PKAN and moderate severity of dystonia. However, two patients with typical PKAN and relatively severe symptoms showed variable responses. CONCLUSIONS: The c.1319G>C (p.R440P) mutation appears to be a founder genotype among Korean patients with PKAN. Furthermore, this study provides additional data for the recent international effort to evaluate the efficacy of pallidal DBS in the treatment of patients with PKAN.Copyright © 2011 The Author(s). European Journal of Neurology © 2011 EFNS.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1468-1331.2011.03589.x" target="_blank" rel="noreferrer noopener">10.1111/j.1468-1331.2011.03589.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).
2012
Chae J H
Cho A
European Journal of Neurology
Hwang H
Hwang Y S
IND
Jeon B S
Ki C S
Kim K J
Kim Y E
Lim B C
Lim Y H
Paek S H
Pallidal deep brain stimulation
surgical intervention
tone and motor problems
Yun J Y
-
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/10.1111/j.1525-1403.2006.00067.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1525-1403.2006.00067.x</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 for dystonia: A meta-analysis
Publisher
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Neuromodulation
Date
A point or period of time associated with an event in the lifecycle of the resource
2006
Subject
The topic of the resource
meta-analysis; Neurosciences & Neurology; dystonia; movement disorders; follow-up; scale; cervical dystonia; deep brain stimulation; electric stimulation therapy; globus-pallidus internus; hemidystonia; meige-syndrome; primary generalized dystonia; Research & Experimental Medicine; severe tardive; spasmodic torticollis; stereotaxic techniques; thalamic-stimulation; tone and motor problems; IND; surgical intervention; deep brain stimulation; BFMDRS
Creator
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Holloway K L; Baron M S; Brown R; Cifu D X; Carne W; Ramakrishnan V
Description
An account of the resource
Objective. To use a meta-analysis on all reported cases of deep brain stimulation (DBS) for dystonia to determine which factors significantly influence outcome. The Burke-Fahn-Marsden (BFM) movement scale, the most reported measure, was chosen as the primary outcome measure for this analysis. Methods. A MEDLINE search identified 137 patients who underwent DBS for dystonia in 24 studies that had individual BFM scores. Individual patient data, including age at onset of dystonia, age at surgery, gender, distribution of dystonia, etiology of dystonia, presence of associated features, abnormality of preoperative imaging, prior stereotactic surgeries, nucleus stimulated, type of anesthesia used, use of physiologic monitoring, type of imaging used for localization, stimulation parameters used, time of response to stimulation, and timing of outcome assessment were entered into an SPSS database for statistical analysis. Results. The mean BFM percentage change (improvement in postoperative score from baseline) was 51.8% (range -34% to 100%). Significantly better outcomes were achieved with stimulation of the globus pallidus internus (GPi) than with stimulation of the posterior portion of the ventral lateral (VLp) nucleus of the thalamus (p = 0.0001). The etiology of the dystonia also had a significant effect on outcomes. Statistically significant improvements in outcomes were seen for all etiologic categories, except encephalitis. Dystonia due to birth injury and encephalitis had significantly worse outcomes when compared to other etiologies. However, there were no significant differences in the outcomes of patients who were DYT1 (DYT1 is the gene associated with the disorder Dystonia Musculorum Deformans) gene positive, DYT1 gene negative, or had pantothenate kinase-associated neurodegeneration (PKAN), tardive dyskinesia, and idiopathic and posttraumatic dystonias. Longer duration of dystonia symptoms correlated negatively with surgical outcome. A regression model using the three variables-stimulation site, etiology of dystonia, and duration of dystonia symptoms-explained 51% of the variance in outcomes. Conclusion. Deep brain stimulation of the GPi provides significant improvement in BFM scores in a variety of dystonic conditions.
Identifier
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<a href="http://doi.org/10.1111/j.1525-1403.2006.00067.x" target="_blank" rel="noreferrer noopener">10.1111/j.1525-1403.2006.00067.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).
2006
Baron M S
BFMDRS
Brown R
Carne W
cervical dystonia
Cifu D X
deep brain stimulation
Dystonia
Electric Stimulation Therapy
Follow-up
globus-pallidus internus
hemidystonia
Holloway K L
IND
meige-syndrome
Meta-Analysis
Movement Disorders
Neuromodulation
Neurosciences & Neurology
primary generalized dystonia
Ramakrishnan V
Research & Experimental Medicine
Scale
severe tardive
spasmodic torticollis
stereotaxic techniques
surgical intervention
thalamic-stimulation
tone and motor problems
-
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/10.1111/j.1365-2788.2006.00889.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1365-2788.2006.00889.x</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
Intellectual and adaptive behaviour functioning in pantothenate kinase-associated neurodegeneration
Publisher
An entity responsible for making the resource available
Journal of Intellectual Disability Research
Date
A point or period of time associated with an event in the lifecycle of the resource
2007
Subject
The topic of the resource
tone and motor problems; IND; trajectory; characteristics; adaptive behavior functioning
Creator
An entity primarily responsible for making the resource
Freeman K; Turner A; Gregory A; Hogarth P; Blasco P; Hayflick S
Description
An account of the resource
BACKGROUND: Pantothenate kinase-associated neurodegeneration (PKAN), an extremely rare autosomal recessive disorder resulting in iron accumulation in the brain, has a diverse phenotypic expression. Based on limited case studies of one or two patients, intellectual impairment is considered part of PKAN. Investigations of cognitive functioning have utilized specific neuropsychological tests, without attention to general intellectual skills or adaptive behaviour. METHODS: Sixteen individuals with PKAN completed measures of global intellectual functioning, and participants or care providers completed measures of adaptive behaviour skills and day-to-day functional limitations. Clinicians provided global ratings of condition severity. RESULTS: Testing with standardized measures documented varied phenotypic expression, with general cognitive skills and adaptive behaviour ranging from high average to well below average. Age of disease onset correlated with measures of intellectual functioning, adaptive functioning and disease severity. CONCLUSIONS: Findings support previously described clinical impressions of varied cognitive impairment and the association between age of onset and impairment. Further, they add important information regarding the natural history of the disease and suggest assessment strategies for use in treatment trials.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1365-2788.2006.00889.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2006.00889.x</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).
2007
adaptive behavior functioning
Blasco P
characteristics
Freeman K
Gregory A
Hayflick S
Hogarth P
IND
Journal Of Intellectual Disability Research
tone and motor problems
Trajectory
Turner A
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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
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
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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
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/ana.20457" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ana.20457</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
Pallidal stimulation improves pantothenate kinase-associated neurodegeneration
Publisher
An entity responsible for making the resource available
Annals of 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
tone and motor problems; IND; surgical intervention; Pallidal Stimulation; dystonia
Creator
An entity primarily responsible for making the resource
Castelnau P; Cif L; Valente E M; Vayssiere N; Hemm S; Gannau A; Digiorgio A; Coubes P
Description
An account of the resource
Pantothenate kinase-associated neurodegeneration (PKAN) causes a progressive generalized dystonia which remains pharmacologically intractable. We performed bilateral internal globus pallidus stimulation in six patients with genetically confirmed PKAN who obtained a major and long-lasting improvement of their painful spasms, dystonia, and functional autonomy. This study shows the benefits of pallidal DBS for the dystonia of PKAN patients.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ana.20457" target="_blank" rel="noreferrer noopener">10.1002/ana.20457</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
Annals Of Neurology
Castelnau P
Cif L
Coubes P
Digiorgio A
Dystonia
Gannau A
Hemm S
IND
Pallidal Stimulation
surgical intervention
tone and motor problems
Valente E M
Vayssiere N