Current and emerging strategies for treatment of childhood dystonia

Abstract

Childhood dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contractions causing twisting and repetitive movements, abnormal postures, or both (Sanger et al. 2003). Dystonia is a devastating neurological condition that prevents the acquisition of normal motor skills during critical periods of development in children. Moreover, it is particularly debilitating in children when dystonia affects the upper extremities such that learning and consolidation of common daily motor actions are impeded. Thus, the treatment and rehabilitation of dystonia is a challenge that continuously requires exploration of novel interventions. This review will initially describe the underlying neurophysiological mechanisms of the motor impairments found in childhood dystonia followed by the clinical measurement tools that are available to document the presence and severity of symptoms. Finally, we will discuss the state-of-the-art of therapeutic options for childhood dystonia, with particular emphasis on emergent and innovative strategies.

Upper‐limb contracture development in children with cerebral palsy: a population‐based study

“…A surprising result of this study at first glance was that children with dyskinetic CP had the highest proportion of contractures as well as the highest risk (crude OR) of developing contractures among the different CP subtypes. This was unexpected because the risk of children with the dyskinetic subtype developing contractures is often regarded as low because of the presence of the large amount of movement and varying muscle tone . However, when adjusting for all predictors (GMFCS and MACS levels, CP subtype, adjusted OR), children with spastic unilateral CP showed the highest OR for contracture development, compared to the other CP subtypes.…”

“…This was unexpected because the risk of children with the dyskinetic subtype developing contractures is often regarded as low because of the presence of the large amount of movement and varying muscle tone. 24,25 However, when adjusting for all predictors (GMFCS and MACS levels, CP subtype, adjusted OR), children with spastic unilateral CP showed the highest OR for contracture development, compared to the other CP subtypes.…”

Scaled Vibratory Feedback Can Bias Muscle Use in Children With Dystonia During a Redundant, 1-Dimensional Myocontrol Task

“…We believed these effects were even more pronounced in children since the inability to acquire new motor skills during early stages of development may further exacerbate their motor disability and limit their social development. Constraint-induced movement therapy and deep brain stimulation are tools that have been used to improve movement 2,7–8 . Furthermore, transcranial direct current stimulation (tDCS) of motor cortex has been used with mixed results 9–11 .…”

The Burke‐Fahn‐Marsden Dystonia Rating Scale is Age‐Dependent in Healthy Children

“…To our best knowledge, BFMDRS scores have never been studied for potential age‐relatedness in children before. We reasoned that forthcoming insight in potential BFMDRS age dependency could provide information for: (1) reliable longitudinal treatment evaluation in young children (such as for longitudinal dystonia databases and for longitudinal evaluation of innovative therapies (such as deep brain stimulation [DBS]); (2) understanding of dystonia progression in different “age‐of‐onset” groups; and (3) adequate phenotypic discrimination between “immature” and “dystonic” motor patterns for adequate interpretation of next‐generation sequencing (NGS) panels …”