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The primary objective of this paper was to evaluate the published evidence of efficacy and safety of botulinum neurotoxin (BoNT) injections in paediatric upper limb hypertonia (PULH). Secondary objectives included the provision of clinical context, based on evidence and expert opinion, in the areas of assessment, child and muscle selection, dosing, and adjunctive treatment. A multidisciplinary panel of authors systematically reviewed, abstracted, and classified relevant literature. Recommendations were based on the American Academy of Neurology (AAN) evidence classification. Following a literature search, 186 potential articles were screened for inclusion, and 15 of these met the criteria and were reviewed. Grade A evidence was found to support the use of BoNT to reach individualized therapeutic goals for PULH. There is grade B evidence (probably effective) for tone reduction following BoNT injections and grade U evidence (inconclusive) for improvement in upper limb (UL) activity and function. BoNT injections were generally found to be safe and well tolerated with the most common side effect identified as a transient decrease in grip strength. [ABSTRACT FROM AUTHOR]

We report the case of an 8-year-old girl who developed progressive generalized dystonia, rendering her unable to walk and sit within months despite medical therapy with dopamine and anti-cholinergic agents. She was found to have a 9q34.1 GAG-deletion, which is known to cause DYTI-dystonia. DYT-1 dystonia is an autosomal dominant condition with incomplete penetrance that usually starts in childhood. It is known to be refractory to pharmacotherapy. Reports on deep brain stimulation in this condition reveal marked benefits of the treatment in the pediatric and adult populations. The patient underwent bilateral stimulation of the internal globus pallidus 18 months after symptom onset. Postoperatively, her clinical status improved significantly as measured by the Burke-Fahn- Marsden dystonia rating scale and the resolution of a unilateral hip dislocation. Normal participation was regained. [ABSTRACT FROM AUTHOR]

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One of the main goals when treating spasticity is to relieve pain and improve function. Intramuscular injection of botulinum toxin type A (BTX-A) has gained widespread acceptance in the treatment of spastic cerebral palsy. Several studies have clearly shown the short-term functional benefit of BTX-A treatment. Information is limited, however, on the efficacy of medium and long-term regimens, using repeated injection of BTX-A. The aim of the present open-label, prospective study was to evaluate functional outcome in children with spastic cerebral palsy after 1 year of treatment with BTX-A, using the Gross Motor Function Measure (GMFM) as a validated outcome measure. Patients (n=25, age 1.5–15.5 years) were treated with BTX-A for adductor spasm (n=12) or pes equinus (n=13). The local effect was evaluated using passive range of motion and modified Ashworth Scale. Apart from a significant improvement in joint mobility and reduction of spasticity compared to pretreatment values (P < 0.01), we demonstrated a significant improvement of gross motor function after 12 months of treatment, with a median gain of 6% in total and goal scores (P < 0.001). An increase in GMFM scores was particularly evident in younger and moderately impaired children (Gross Motor Function Classification System level III). Whether the observed improvement in gross motor function in children with cerebral palsy is specifically related to therapy with BTX-A or represents at least in part the natural course of motor development still needs clarification. [ABSTRACT FROM AUTHOR]