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http://dx.doi.org/10.1136/archdischild-2020-319878DOI Listing

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Objectives: Intrathecal baclofen (ITB) therapy is well documented as an effective treatment option for severe spasticity. Before ITB implantation, trials are conducted to evaluate efficacy, safety, and candidate suitability. While many centers conduct ITB trials, appropriate physical assessment has not been fully established.

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Purpose Of Review: Baclofen, a muscle relaxant that reduces the release of excitatory neurotransmitters in the pre-synaptic neurons stimulating inhibitory neuronal signals in post-synaptic neurons, has been around for over 5 decades. Baclofen is used primarily for spasticity and since 1982, has had a role as an intrathecal agent. In the present investigation, we review research trends and updates on safety and efficacy of intrathecal baclofen (ITB) pumps.

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Objective: Intraventricular baclofen (IVB) administration is used for the treatment of secondary dystonia associated with cerebral palsy (CP), but it has not been reported as a first-line infusion technique for spasticity. In this study, the authors report outcomes of patients with mixed or isolated spasticity treated with IVB administration.

Methods: A retrospective analysis was performed of consecutive patients treated with IVB between 2019 and 2023.

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Purpose: While surgical intervention of scoliosis in cerebral palsy (CP) patients has shown notable improvements in quality of life, the high risk of post-operative complications in CP patients necessitates careful preoperative optimization. A preoperative multidisciplinary (Multi-D) pathway at our tertiary pediatric hospital in effect since 2014 led to a significant reduction in mortality at one year. However, such a strategy delays surgery, potentially increasing the risk of curve progression.

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Background: While the symptoms of Huntington's disease (HD) typically first appear around the age of 40 years, 5%-10% of patients experience symptoms before the age of 21 years, in which case it is classified as juvenile Huntington's disease (JHD). JHD poses a unique clinical problem, as affected patients experience rapid deterioration in their quality of life as the motor manifestations of the disease become overwhelming. Medical treatment options for HD are sparse, and the only Food and Drug Administration-approved medication for the treatment of HD is the VMAT-2 inhibitor tetrabenazine.

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