Identifying Upper Extremity Features of Dystonia in People with Cerebral Palsy (P5-9.012)

Abstract

<h3>Objective:</h3> To identify expert clinician-cited features of upper extremity dystonia in people with cerebral palsy (CP). <h3>Background:</h3> Dystonia in CP is debilitating yet underdiagnosed, particularly when co-existent with spasticity. Subjective expert clinician consensus remains the diagnostic gold standard, but the specific features leading experts to make a dystonia diagnosis remain unclear. <h3>Design/Methods:</h3> To determine expert clinician-cited features of dystonia, we performed a conventional content analysis of consensus-building discussions between three pediatric movement disorder specialists as they evaluated upper extremity dystonia severity in 26 neurologic exam videos of seated subjects with CP and spasticity. <h3>Results:</h3> 45.8% of discussion codes related to body region, actions, movement features, laterality, or examination features (with the remainder on severity score deliberation and dystonia diagnostic difficulty). Experts cited involvement of the “shoulder” significantly more frequently as dystonia severity increased (<i>p</i>&lt;0.005, chi-squared test) as opposed to the “hand” which was cited less frequently (<i>p</i>&lt;0.0005, chi-squared test). “Mirror movements” were cited significantly more frequently in videos with no or mild dystonia (p&lt;0.005, chi-squared test). Though “unilateral” was the top cited laterality code (72%) and “variability over time” was the top cited movement feature code (34%), neither were significantly associated with dystonia severity (p=0.09, p=0.052 respectively, chi-squared test), in contrast to our previous lower extremity dystonia analysis findings. In videos where diagnostic consensus was reached only after consensus-building discussion (4/26 videos), the repetitive hand “open/close” exam maneuver was the top cited exam maneuver (28%) and was referred to significantly more frequently than for videos where consensus was reached prior to any discussion (p&lt;0.005). <h3>Conclusions:</h3> Experts use distinct movement features to diagnose upper extremity dystonia in people with CP and spasticity. Efforts like this can be used to target examinations and codify the defining features of dystonia in people with CP, thus helping to facilitate dystonia diagnosis. <b>Disclosure:</b> Dr. Gilbert has nothing to disclose. Ms. Gandham has nothing to disclose. Dr. Pearson has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Teva Pharmaceuticals. The institution of Dr. Pearson has received research support from NIH. Dr. Ueda has nothing to disclose. Dr. Aravamuthan has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurocrine Biosciences. An immediate family member of Dr. Aravamuthan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for SK Life Science, Inc.. Dr. Aravamuthan has received research support from National Institute of Neurological Disorders and Stroke.