ABSTRACT
Background and Objectives: Dystonia in cerebral palsy (CP) is debilitating and common, but underdiagnosed, especially when coexistent with spasticity. With dedicated research-based assessment, dystonia is found in most people with spastic CP but is only clinically diagnosed in the minority. To begin addressing the high rates of dystonia underdiagnosis in this population, we determined the key feature experts use to assess upper extremity dystonia in people with spastic CP. Methods: In this prospective cohort study, 3 pediatric movement disorder specialists assessed upper extremity dystonia in neurologic examination videos of people with spastic CP and isolated periventricular leukomalacia (PVL) on brain MRI (i.e., those with a brain injury pattern typical for spastic CP). Dystonia severity was rated using the 10-point Global Dystonia Severity Rating Scale, first by each expert independently and then again after consensus-building discussion. Conventional content analysis of these discussions revealed salient features ("codes") that experts used to assess upper extremity dystonia. Code frequency distributions were compared between dystonia severity categories using χ2 tests. Results: We identified 96 people with spastic CP with isolated PVL on brain MRI seen in the St. Louis Children's Hospital CP Center between 2005 and 2018. Of them, 26 people were able and willing to be recorded while doing a standardized set of upper extremity examination maneuvers (age 4-25 years; 28% nonambulatory, 77% White). When assessing their videos, experts cited the "hand" less often and "shoulder" more often with increasing dystonia severity (p < 0.005, χ2 test). "Mirror movements" and the "hand open/close" examination maneuver were cited significantly more frequently in videos when experts were attempting to distinguish between no dystonia and mild dystonia (p < 0.005). Discussion: Expert clinicians use distinct movement features to assess upper extremity dystonia in people with spastic CP and PVL. Attention to involuntary shoulder (vs hand) movements can help gauge dystonia severity. Differentiation between mirror movements and dystonia, particularly during the hand open/close examination maneuver, may help identify mild dystonia. These results can help guide upper extremity dystonia assessment in people with spastic CP, thus potentially helping mitigate dystonia underdiagnosis.
ABSTRACT
Dystonia in cerebral palsy (DCP) is a common, debilitating, but understudied condition. The CP community (people with CP and caregivers) is uniquely equipped to help determine the research questions that best address their needs. We developed a community-driven DCP research agenda using the well-established James Lind Alliance methodology. CP community members, researchers, and clinicians were recruited through multiple advocacy, research, and professional organizations. To ensure shared baseline knowledge, participants watched webinars outlining our current knowledge on DCP prepared by a Steering Group of field experts (cprn.org/research-cp-dystonia-edition). Participants next submitted their remaining uncertainties about DCP. These were vetted by the Steering Group and consolidated to eliminate redundancy to generate a list of unique uncertainties, which were then prioritized by the participants. The top-prioritized uncertainties were aggregated into themes through iterative consensus-building discussions within the Steering Group. 166 webinar viewers generated 67 unique uncertainties. 29 uncertainties (17 generated by community members) were prioritized higher than their randomly matched pairs. These were coalesced into the following top 10 DCP research themes: (1) develop new treatments; (2) assess rehabilitation, psychological, and environmental management approaches; (3) compare effectiveness of current treatments; (4) improve diagnosis and severity assessments; (5) assess the effect of mixed tone (spasticity and dystonia) in outcomes and approaches; (6) assess predictors of treatment responsiveness; (7) identify pathophysiologic mechanisms; (8) characterize the natural history; (9) determine the best treatments for pain; and (10) increase family awareness. This community-driven research agenda reflects the concerns most important to the community, both in perception and in practice. We therefore encourage future DCP research to center around these themes. Furthermore, noting that community members (not clinicians or researchers) generated the majority of top-prioritized uncertainties, our results highlight the important contributions community members can make to research agendas, even beyond DCP.
