Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit
Title
Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit
Creator
Puertas MV; de Noriega Echevarria I; Errasti VI; Perez MMA; Alba RM
Identifier
Publisher
Neurologia
Date
2024
Subject
alert; baclofen/dt [Drug Therapy]; baclofen/po [Oral Drug Administration]; baclofen/pv [Special Situation for Pharmacovigilance]; baclofen/tl [Intrathecal Drug Administration]; dystonia/dt [Drug Therapy]; palliative therapy; pediatrics; spasticity/dt [Drug Therapy]; Gross Motor Function Measure; STATA v16.0; adolescent; adult; antibiotic agent/dt [Drug Therapy]; antibiotic agent/iv [Intravenous Drug Administration]; article; benzodiazepine derivative/dt [Drug Therapy]; benzodiazepine derivative/po [Oral Drug Administration]; benzodiazepine derivative/pv [Special Situation for Pharmacovigilance]; botulinum toxin/dt [Drug Therapy]; brain damage; catheter; cerebral palsy; child; clinical article; clobazam/dt [Drug Therapy]; clonazepam/dt [Drug Therapy]; congenital malformation; continuous infusion; descriptive research; diazepam/dt [Drug Therapy]; disease classification; drug dose increase; drug dose reduction; enzyme deficiency; female; glutaric aciduria; glutaric aciduria type 1; human; implantation; intrathecal baclofen pump/am [Adverse Device Effect]; intrathecal pump; male; medical device malfunction/co [Complication]; meningitis/dt [Drug Therapy]; nerve degeneration; pantothenate kinase/ec [Endogenous Compound]; poly malformative syndrome; preschool child; reservoir; retrospective study; school child; syndrome; tizanidine/dt [Drug Therapy]; tizanidine/po [Oral Drug Administration]; tizanidine/pv [Special Situation for Pharmacovigilance]; trihexyphenidyl/dt [Drug Therapy]; trihexyphenidyl/po [Oral Drug Administration]; trihexyphenidyl/pv [Special Situation for Pharmacovigilance]; young adult
Description
Introduction: Patients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered. <br/>Material(s) and Method(s): Descriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021. <br/>Result(s): IBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV-V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period "honeymoon". Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3-25.8). <br/>Conclusion(s): IBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders.<br/>Copyright © 2022 Sociedad Espanola de Neurologia
Rights
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Citation List Month
November List 2024
URL Address
Collection
Citation
Puertas MV; de Noriega Echevarria I; Errasti VI; Perez MMA; Alba RM, “Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit,” Pediatric Palliative Care Library, accessed February 8, 2025, https://pedpalascnetlibrary.omeka.net/items/show/19788.