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Dublin Core
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Title
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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
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URL Address
<a href="http://doi.org/10.1016/j.ejpn.2017.09.003" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ejpn.2017.09.003</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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Intrathecal baclofen treatment an option in X-linked adrenoleukodystrophy
Publisher
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European Journal of Paediatric Neurology
Date
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2018
Subject
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quality of life; pain; limited mobility; priority journal; school child; spasticity; fatigue; clinical examination; human; article; child; male; clinical article; dystonia; case report; 1309378-01-5 (botulinum toxin A); 1638949-86-6 (botulinum toxin A); 1800016-51-6 (botulinum toxin A); 93384-43-1 (botulinum toxin A); 1134-47-0 (baclofen); adrenoleukodystrophy/dt [Drug Therapy]; adrenoleukodystrophy/su [Surgery]; baclofen/dt [Drug Therapy]; baclofen/po [Oral Drug Administration]; baclofen/tl [Intrathecal Drug Administration]; X chromosome linked disorder/dt [Drug Therapy]; X chromosome linked disorder/su [Surgery]; Addison disease; adrenoleukodystrophy/dt [Drug Therapy]; balance disorder; behavior change; bladder dysfunction; botulinum toxin A; clonus; diplopia; drug dose increase; hearing disorder; hyperpigmentation; intrathecal pump; leukodystrophy; range of motion; strabismus; urinary catheter; visual disorder; X chromosome linked disorder/dt [Drug Therapy]; tone and motor problems; X-linked adrenoleukodystrophy; pharmacologic interventions; intrathecal baclofen; baclofen
Creator
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Hjartarson H T; Ehrstedt C; Tedroff K
Description
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Background X-linked adrenoleukodystrophy (X-ALD) is a genetic peroxisomal disorder associated with tissue accumulation of very long chain fatty acids (VLCFAs). In approximately one third of affected males, this causes progressive and irreversible damage to the brain white matter. Progress is often rapid with upper motor neuron damage leading to severe spasticity and dystonia. The increased muscle tone is frequently difficult to alleviate with oral drugs. Here, we describe two patients with X-ALD who have received treatment with intrathecal baclofen pumps (ITB). Case study Both boys had a rapidly progressive cerebral form of the disorder resulting, among other things, in escalating spasticity and dystonia causing severe pain, dramatically reducing their quality of life. Both were treated with a variety of oral medications without adequate relief. Both patients tolerated ITB surgery without complications and the positive clinical effects of treatment with ITB became clear in the following weeks and months, with significantly reduced muscle tone, less pain and better sleep. Moreover, general caretaking became easier. Conclusion The treatment of spasticity and dystonia in these patients is difficult partly due to the relentless nature of this progressive disorder. In our two patients, ITB has been effective from both a symptomatic and palliative perspective. We recommend that such treatment be considered as an early option for increased muscle tone in boys with the cerebral form of X-ALD.
Identifier
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<a href="http://doi.org/10.1016/j.ejpn.2017.09.003" target="_blank" rel="noreferrer noopener">10.1016/j.ejpn.2017.09.003</a>
Rights
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
1134-47-0 (baclofen)
1309378-01-5 (botulinum toxin A)
1638949-86-6 (botulinum toxin A)
1800016-51-6 (botulinum toxin A)
2018
93384-43-1 (botulinum toxin A)
Addison disease
adrenoleukodystrophy/dt [Drug Therapy]
adrenoleukodystrophy/su [Surgery]
Article
baclofen
Baclofen/dt [drug Therapy]
baclofen/po [Oral Drug Administration]
baclofen/tl [Intrathecal Drug Administration]
balance disorder
behavior change
bladder dysfunction
botulinum toxin A
Case Report
Child
Clinical Article
clinical examination
clonus
diplopia
drug dose increase
Dystonia
Ehrstedt C
European Journal of Paediatric Neurology
Fatigue
hearing disorder
Hjartarson H T
Human
hyperpigmentation
intrathecal baclofen
intrathecal pump
Leukodystrophy
limited mobility
Male
Pain
pharmacologic interventions
Priority Journal
Quality Of Life
Range of Motion
School Child
Spasticity
strabismus
Tedroff K
tone and motor problems
urinary catheter
visual disorder
X chromosome linked disorder/dt [Drug Therapy]
X chromosome linked disorder/su [Surgery]
X-linked adrenoleukodystrophy