Developing a Device for Guiding Therapy in Ataxia and Imbalance

Funded by the Medical Research Future Funds (MRFF) under the Biomedical Translation Bridge (BTB) Program

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Ataxia is a disabling movement disorder resulting from cerebellar dysfunction. There is a medical and commercial need for accurate measurement of ataxia to aid the development of these therapies and for their continuing use in ongoing treatment. In particular, objective assessment of Friedreich ataxia (FA), the most common of the genetic ataxias, is crucial for developing new medications and therapies. We are developing standard prototypes for specialized data loggers and algorithms that address this need. This project at Deakin University’s Networked Sensing & Biomedical Engineering Research Lab is supported by the Medical Research Future Fund (MRFF) – Biomedical Translation Bridge (BTB) program. The Florey Institute of Neuroscience and Mental Health and The University of Melbourne lead the program and partner with Deakin University and Murdoch Children’s Research Institute.

Our aim in this project is to convert our algorithms to medical-grade software and build data loggers to regulatory standards. In conclusion, a start-up company could consider approaching customers who require objective measurement in clinical trials.

This is an initiative of the MRFF, operated by MTPConnect in partnership with three BTB Venture Partners, BioCurate, Medical Device Partnering Program (MDPP) and UniQuest, and the Bridge and BridgeTech Programs administered by Queensland University of Technology (QUT). The BTB Program aims to nurture the translation of new therapies, technologies, and medical devices through to the proof of concept stage. Activities supported by the BTB program include hit-lead optimization and validation studies (therapeutic focused projects), prototype development and product testing (medical device focused projects), as well as regulatory support, pre-clinical and clinical trial activity.

Cerebellar Ataxia

Cerebellar Ataxia refers to the disturbance in movement resulting from cerebellar dysfunction. It manifests as inaccurate movements with delayed onset and overshoot, especially when movements are repetitive or rhythmic. Identification of ataxia is integral to the diagnosis and assessment of severity and is important in monitoring progression and improvement. Ataxia is identified and assessed by clinicians observing subjects perform standardized movement tasks that emphasize ataxic movements. There are many neurological tests designed to analyze motor impairments in different domains (such as an upper limb, lower limb, gait, balance, and speech). Clinicians follow scoring protocols to record the severity of affected individuals for each domain test.

Friedreich Ataxia

Friedreich ataxia (FA), the most common of the inherited ataxias, is a degenerative disease that progressively affects walking and other functions leading to significant impairment associated with a shortened lifespan. It is important to monitor the progression of ataxia over periods of time for clinical and therapeutic interventions [M1]. The innovation is the development of the Ataxia Instrumented Measurement System (AIM) which consists of task-related data loggers that are equipped with the Internet-of-Things (IoT) system (sensors, a microprocessor, memory, and WIFI that can sense relevant movement, process the data, and send it by our own App to a smartphone). By the same App, the data is sent to the cloud where our algorithms operate on the data to produce scores that describe the presence and severity of ataxia. These scores are available on the smartphone App in real-time.

There are three AIM tasks that emulate simple tasks of daily living and reflect the complexity of living with ataxia. The Ataxia Instrumented Measurement Systems consists of three devices: Spoon (AIM-S), Cup (AIM-C) and the Pendant (AIM-P) :

DEA-2131_Ataxia_Devices_Colour_Scheme_3_Nov_2021

Ataxia Instrumented Measurement Systems-Spoon (AIM-S)

The AIM-S is a logger for the AIM.  It is a spoon, equipped with the IoT-based system, which when linked via our smartphone-based app to the cloud-based algorithms can provide a score of ataxia.  This score reflects the effect of ataxia on the process of using a spoon to take food from a bowl to the mouth and then return the spoon to the table.

Ataxia Instrumented Measurement Systems-Cup (AIM-C)

The AIM-C is a logger for the AIM.  It is a canister that a subject can use to simulate the act of drinking from a cup. The canister contains the IoT-based system, which measures the movement as well as the pressure being applied to the canister during simulated drinking. When linked via our smartphone-based app to the cloud-based algorithms it can provide a score of ataxia during an activity commonly undertaken as an activity of daily living.

Ataxia Instrumented Measurement Systems-Pendant (AIM-P)

The AIM-P is a logger for the AIM.  It is a single kinematic sensor attached to the wearer’s chest to measure balance during a simple standing test.  When linked via our smartphone-based app to the cloud-based algorithms it can provide a score of ataxia during an activity commonly undertaken as an activity of daily living.

Our Team

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Professor Malcolm Horne

Chief Investigator

Malcolm Horne has appointments as a Consultant Neurologist specializing in Parkinson’s disease at the St. Vincent’s Hospital, a Senior Principle Research Fellow at the Florey Institute of Neurosciences and Mental Health, and an Adjunct Professor with the Department of Medicine, University of Melbourne. His past appointments have included the Deputy Director and NHMRC Practitioner Fellow at the Florey Institute of Neurosciences and Mental Health, the Founder, CSO, and CEO of Global Kinetics, a Professor with the Department of Medicine, Monash University, and the Director of the NeurologyMonash Medical Centre. His research interests relate to various facets ofParkinson’s disease, dopamine, and frontal lobe function. His current research interest is in using machine learning and wearable sensors to better measure and understand various disorders of movement.

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Professor Pubudu N. Pathirana

Chief Investigator

Pubudu N. Pathirana (SM’08) received the B.E. (Hons.) degree in electrical engineering and the B.Sc. degree in mathematics, and the Ph.D. degree in electrical engineering from The University of Western Australia, in 1996 and 2000, respectively, all sponsored by the government of Australia on EMSS and IPRS scholarships. He was a Post-Doctoral Research Fellow at Oxford University, U.K., a Research Fellow at the School of Electrical Engineering and Telecommunications, University of New South Wales, Sydney, Australia, and a Consultant of the Defence Science and Technology Organization (DSTO), Australia, in 2002. In 2009, he was a Visiting Associate Professor at Yale University. He is currently a Professor with the School of Engineering and the Director of the Networked Sensing and Control Research Group, Deakin University, Geelong, Australia. His current research interests include bio-medical assistive device design, human motion capture, mobile/wireless networks, rehabilitation robotics, and radar array signal processing.

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Ass. Professor Louise Corben

Chief Investigator

Associate Professor Louise Corben is a MRFF Next Generation Career Development Fellow based in the Bruce Lefroy Centre in the Murdoch Childrens Research Institute. In this role she also coordinates the Friedreich ataxia clinical research programme at the Bruce Lefroy Centre and the dedicated Friedreich ataxia clinic at Monash Medical Centre, Clayton.

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Dr. David Szmulewicz

Chief Investigator

David Szmulewicz received the Ph.D. degree from the University of Melbourne. He is a Neurologist, a Neurotologist, and a Medical researcher. He is the Head of balance disorders and ataxia service with the Royal Victorian Eye andEarHospital, the Cerebellar Ataxia Clinic, and the Alfred Hospital, and a Neurologist with the Monash Medical Centre, Friedreich’s Ataxia Clinic. He is also the Co-Director of The Australian Temporal Bone Bank that aims to facilitate pathological investigation of hearing and balance diseases. He is a Lead Investigator on research defining a novel ataxia–Cerebellar Ataxia With Neuropathy and Vestibular Areflexia Syndrome (CANVAS), a project to develop objective ataxia metrics and an objective bedside test of imbalance–the video VVOR. His clinical and research interests include balance disorders that affect the vestibular system, cerebellum, and the combination of the two.

Project Manager

Srinivas Shesham

Research Engineers

Bipasha Kashyap
Chandima Kolambahewage
Ha Tran
Khoa Nguyen
Lahiru Abeysekara
Pasindu Epa
Ragil Krishna
Sandini Anuradha
Sean Starick
Tony Ngo




Publications

POSTURAL BALANCE:

  1. Nguyen, N., Phan, D., Pathirana, P., Horne, M., Power, L. and Szmulewicz, D., 2018. Quantification of Axial Abnormality Due to Cerebellar Ataxia with Inertial Measurements. Sensors, 18(9), p.2791. [J] 
  2. Ngo, T., Abeysekara, L.L., Pathirana, P.N., Horne, M.K., Power, L., and Szmulewicz, D.J., 2020. A Comparative Severity Assessment of Impaired Balance Due to Cerebellar Ataxia Using Regression Models. In 2020 42nd Annual International Conference of the IEEE Engineering in Medicine and Biology Society (EMBC), (pp. 4571-4574), IEEE[C]

DEVICES:

Spoon

  1. Nguyen, K.D., Corben, L.A., Pathirana, P.N., Horne, M.K., Delatycki, M.B. and Szmulewicz, D.J., 2019, July. An Instrumented Measurement Scheme for the Assessment of Upper Limb Function in Individuals with Friedreich Ataxia. In 2019 41st Annual International Conference of the IEEE Engineering in Medicine and Biology Society (EMBC) (pp. 317-320). IEEE. [C] 
  2. Nguyen, K.D., Corben, L.A., Pathirana, P.N., Horne, M.K., Delatycki, M.B. and Szmulewicz, D.J., 2020. The Assessment of Upper Limb Functionality in Friedreich Ataxia via Self-Feeding Activity. IEEE Transactions on Neural Systems and Rehabilitation Engineering, 28(4), pp. 924 – 933. [J]
  3. Nguyen, K.D., Corben, L.A., Pathirana, P.N., Horne, M.K., Delatycki, M.B. and Szmulewicz, D.J., 2020, July. Assessment of Disease Progression in Friedreich Ataxia using an Instrumented Self Feeding Activity. In 2020 42nd Annual International Conference of the IEEE Engineering in Medicine and Biology Society (EMBC) (pp. 3827-3830). IEEE. [C] 
  4. Corben, L.A., Nguyen, K.D., Pathirana, P.N., Horne, M.K., Szmulewicz, D.J., Roberts, M. and Delatycki, M.B., 2021. Developing an Instrumented Measure of Upper Limb Function in Friedreich Ataxia. The Cerebellum, pp.1-9. [J]

Cup

  1. Krishna, R., Pathirana, P.N., Horne, M., Power, L. and Szmulewicz, D., 2020, July. Quantitative Assessment of Friedreich Ataxia through the Self-Drinking Activity. In 2020 42nd Annual International Conference of the IEEE Engineering in Medicine and Biology Society (EMBC) (pp. 820 – 823). IEEE.[C]
  2. Krishna R., Pathirana P. N., Horne M. K., Corben L. A., and Szmulewicz D. J., "Quantitative Assessment of Friedreich Ataxia via Self-Drinking Activity, 2021. IEEE Journal of Biomedical and Health Informatics, vol. 25, no. 6, pp. 1985-1996, June, doi: 10.1109/JBHI.2021.3069007

FULL BODY:

  1. Kashyap, B., Phan D., Pathirana, P.N., Horne, M., Power, L. and Szmulewicz, D., (in press), 2020. Objective Assessment of Cerebellar Ataxia: A Comprehensive and Refined Approach. Scientific reports. [J]
  2. Kashyap, B., Phan D., Pathirana, P.N., Horne, M., Power, L. and Szmulewicz, D., 2020, July. A Sensor-Based Comprehensive Objective Assessment of Motor Symptoms in Cerebellar Ataxia. In 2020 42nd Annual International Conference of the IEEE Engineering in Medicine and Biology Society (EMBC) (pp. 816-819). IEEE[C]
  3. Abeysekara, L.L., Tran, H., Pathirana, P.N., Horne, M., Power, L. and Szmulewicz, D., 2020, July. Multi-domain Data Capture and Cloud Buffered Multimodal Evaluation Platform for Clinical Assessment of Cerebellar Ataxia. In 2020 42nd Annual International Conference of the IEEE Engineering in Medicine and Biology Society (EMBC) (pp. 5640-5643), IEEE[C]