Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit

Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit

Puertas MV; de Noriega EI; Errasti VI; Perez MMA; Alba RM

Neurologia

2024

Humans; Child; Child Preschool; Female; Male; Retrospective Studies; Adolescent; Infant; Palliative Care; Muscle Relaxants Central; Muscle Relaxants Central/ad [Administration & Dosage]; Cerebral Palsy/dt [Drug Therapy]; Baclofen; Dystonia; Injections Spinal; Muscle Spasticity; Baclofen/ad [Administration & Dosage]; Baclofen/tu [Therapeutic Use]; Dystonia/dt [Drug Therapy]; Muscle Relaxants Central/tu [Therapeutic Use]; Muscle Spasticity/dt [Drug Therapy]; Pantothenate Kinase-Associated Neurodegeneration/dt [Drug Therapy]

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. Materials and methods: 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. Results: 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). Conclusions: 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.

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