An Evaluation of Gait, Parkinsonian Features, and Adaptive Behaviours in Adults with Dravet Syndrome
Abstract number :
3.083
Submission category :
2. Translational Research / 2A. Human Studies
Year :
2021
Submission ID :
1826298
Source :
www.aesnet.org
Presentation date :
12/6/2021 12:00:00 PM
Published date :
Nov 22, 2021, 06:53 AM
Authors :
Arunan Selvarajah, MSc (candidate) - University Health Network; Carolina Gorodetsky, MD - Fellow, Movement Disorders, University Health Network; Paula Marques, MD - Fellow, Neurology, University Health Network; Quratulain Zulfiqar Ali - Neurology - University Health Network; Ilakkiah Chandran - University Health Network; Tara Sadoway - University Health Network; Fabio Nascimento, MD - University Health Network; Anne Berg, PhD - Research Professor, Neurology, Ann & Robert H. Lurie Children’s Hospital of Chicago; Mary Pat McAndrews, PhD, C.Psych - Psychology - University Health Network; Alfonso Fasano, MD, PhD - Movement Disorders - University Health Network; Danielle Andrade, MD, MSc - Adult Neurologist, Neurology, University Health Network
Rationale: Dravet Syndrome (DS) is a rare, treatment-resistant, epileptic encephalopathy that usually starts in the first year of life. The intractable seizures adversely impair cognition, motor, and lead to gradual and chronic loss of cognitive abilities over time. There are a couple of studies that have identified parkinsonian features in DS. Our objective was to evaluate gait, parkinsonian features, and adaptive behaviour skills in our DS patients.
Methods: Gait analysis data was extracted from a gait mat built with sensors, in combination with the ProtoKinetics software and two cameras that were 90 degrees apart (sagittal and coronal planes). Clinical mUPDRS scores were assigned to resting tremors, facial expression, arising from a chair, gait, freezing of gait, posture, and body bradykinesia. Regression analyses were performed for the gait and mUPDRS data. We performed a longitudinal observational study, examining the Vineland Adaptive Behaviour Scales 2nd edition (VABS-II) in 2014 and again in 2019. For statistical analysis, we performed the Wilcoxon Signed Rank Test, to detect statistically significant differences.
Results: Gait analysis showed young DS adults performed worse than an average healthy 62-year old control group in most gait parameters. Parkinsonian features got worse with age. Initially, we observed statistically insignificant decreases in communication and daily living skills, and increase in socialization skills. Removal of contraindicated medications between the first and second administration of VABS-II did not display stabilization in gross motor skills.
Conclusions: For clinical relevance, it is very important to determine if DS patients experience a neurodegenerative process. So far, these patients are seen and treated as having a neurodevelopmental epileptic encephalopathy. However, understanding how their adult brains age is of utmost importance to proper patient care.The study findings suggest performing similar assessments as early as possible, to allow for a holistic approach to care and use of support services.
Funding: Please list any funding that was received in support of this abstract.: Dravet Syndrome Foundation and Dravet Canada.
Translational Research