The effect of home based exergame balance training on pediatric ataxia.

Early Onset Ataxia Balance Exergames

prof. dr. ir. N.M. Maurits
Dr. D.A. Sival
C. Lamoth
R. Brandsma

Nature of the research:
explorative pilot study (preceding an MD PhD study project)

Fields of study:
movement sciences neurology rehabilitation

Background / introduction
Early Onset Ataxia (EOA) concerns a group of rare children with heterogeneous, (mostly) degenerative neurologic diseases with ataxia as the leading symptom. Children with EOA reveal motor performances that lack sufficient coordination of arm-, leg-, trunk-, eye- and speech- muscles, resulting in problematic balance, limb coordination and speech.1,2 To date, there is no medication available to ameliorate the progression of ataxia.3 Recent publications in patients have indicated that training with exergames (virtual video-games) are beneficial for their coordinative functions.4-9 It is still unknown whether this effect is attributable to improved axial muscle force (providing trunk stability), or to improved cerebellar coordination. Furthermore, before clinical application in EOA children, insight in the optimal exergame strategy for the specific phenotype of the patient is needed, first.
The UMCG has recently developed an ice skating exergame, during which the patient can induce virtual skating movements in a puppet (visible on a screen), by accurately shifting his/her body balance.7,8 In a phenotypically well-circumscribed ambulant subgroup of EOA children, we aim to evaluate the effects of this skating exergame on: a/ ataxia parameters (by measurements with non-invasive, light weight inertial measurement units (IMUs)10, clinical scores with ataxia rating scales (SARA)11 and pediatric balance scale12, b/ muscle parameters (muscle force assessment by dynamometry13 and muscle ultrasound imaging14,15), c/ pre-defined improvement of coordinative functions (goal attainment scores16).
Research question / problem definition
Can the skating exergame improve coordinative function in a phenotypically well-circumscribed ambulant subgroup of EOA children? And if so, is the outcome attributable to improvement of skeletal muscle force, or to improved cerebellar functioning?
During a period of six weeks, five mildly ataxic children and five healthy, age-matched control children will play the skating exergame 3 times a week during 30 minutes in their own home environment. During the game, whole body parameters will be recorded by a Kinect-system, that can quantify balance performances every minute during each 30 minute training session. At the outpatient clinics, we will obtain neurologic data regarding: a. ataxia rating scale scores (walking, posture, limb kinetics), b. muscle force data (i.e. dynamometry of trunk and limb muscles before, during and at the end of the training period), c. muscle ultrasound parameters (of limb muscles before and after the training period).

After the six weeks, we will analyze the data and prepare a manuscript. The primary objective of this pilot study is to evaluate the feasibility of the ice-skating exergame training in ataxic children. This study serves as a pilot for sample size calculation and study design of a collaborative MD PhD study project to evaluate the clinical application of exergame training in children with EOA.

In collaboration with the multidisciplinary team (pediatric neurology-movement disorders; neuroengeneering; movement & medical sciences), the student participates in:
1. pediatric support and surveillance during the exergame training period (both patients and controls), 2. recording and assessment of the neurologic parameters (a, b, c), 3. analysis of the kinetic data, 4. evaluation of the outcome parameters, 5. if applicable and requested by the student (in case of satisfactory and substantial contribution), preparation of the manuscript and/or writing of a MD PhD proposal.
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2. Bodranghien F, Bastian A, Casali C, et al. Consensus paper: Revisiting the symptoms and signs of cerebellar syndrome. Cerebellum. 2016;15(3):369-391.
3. Brandsma R, Kremer HP, Sival DA. Riluzole in patients with hereditary cerebellar ataxia. Lancet Neurol. 2016;15(8):788-4422(16)00131-9.
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5. Schatton C, Synofzik M, Fleszar Z, Giese MA, Schols L, Ilg W. Individualized exergame training improves postural control in advanced degenerative spinocerebellar ataxia: A rater-blinded, intra-individually controlled trial. Parkinsonism Relat Disord. 2017;39:80-84.
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8. Soancatl Aguilar V, Lamoth CJC, Maurits NM, Roerdink JBTM. Assessing dynamic postural control during exergaming in older adults: A probabilistic approach. Gait Posture. 2017;60:235-240.
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10. Mannini A, Martinez-Manzanera O, Lawerman TF, Sival DA et al. Automatic classification of gait in children with early-onset ataxia or developmental coordination disorder and controls using inertial sensors. Gait Posture. 2017;52:287-292.
11. Lawerman TF, Brandsma R, Burger H, Burgerhof JGM, Sival DA, the Childhood Ataxia and Cerebellar Group of the European Pediatric Neurology Society. Age-related reference values for the pediatric scale for assessment and rating of ataxia: A multicentre study. Dev Med Child Neurol. 2017;59(10):1077-1082.
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