Assessment of Safety and Acute Effects of a Lower-limb Powered Dermoskeleton in Patients With Neuromuscular Disorders (Exo-KGO1)

The aims of the current study are as follow:

i) Evaluate the safety, usability, and acute efficiency of a programmable ambulation exoskeleton (KeeogoTM Dermoskeleton System, B-Temia Inc., Quebec, Canada) in patients with neuromuscular disorders, ii) Elaborate recommendations regarding usability criteria for safe and efficient use the device in patients with neuromuscular disorders (e.g. type and severity of patient's functional deficits), iii) generate necessary data to foresee a future study involving a home use of the device and assessment of long-term benefits.

Study Overview

• Status: Completed
• Conditions: Muscular Dystrophies; Mitochondrial Myopathies; Glycogen Storage Disease; Idiopathic Inflammatory Myopathies; Congenital Myopathy
• Intervention / Treatment: Device: Lower-limb powered dermoskeleton

Detailed Description

Patients with neuromuscular disorders display different type of symptoms depending on the type of pathology. Diseases like facioscapulohumeral dystrophy (FSHD), limb-girdle muscular dystrophy (LGMD2b), sporadic inclusion body myositis (SIBM) or Pompe disease (PD) are characterized by severe muscle weakness leading to reduced functional capacities. This leads to a dramatic decrease of quality of life (e.g. reduced autonomy/participation, social isolation, depression) associated with increased fall risk and complications (e.g. trauma, cardio-vascular issues, trauma, chronic pain, loss of bone mass, and weight gain).

However, a residual of level of strength and residual function may be maintained over years, even at severe disease stages allowing transfers and ambulation. However, the maintenance of this type of activity is often associated with substantial compensatory movements, leading to high load on joints, orthopedic complications, and high fall-risk.

In contrast to traditional passive assistive devices such as orthoses, powered assistive devices, frequently termed dermo- or exoskeletons, have a very high potential for compensating muscle weakness and regain mobility and independence. Devices such as the ReWalk® or the Indego® use rigid structures, in parallel to the user's legs, and electric motors to stabilize the human against gravity during standing and walking. Thus far, these systems have been used mostly in clinical environments for gait rehabilitation in neurological conditions (e.g. spinal cord injury, post-stroke syndrome). Their weight, which can range from 13 to 48 kg, can make them difficult to use and transport, thus limiting their applicability beyond clinical environments. However, for assistive devices to be used in everyday life, they must provide assistance across activities of daily living (ADLs) in an unobtrusive manner.

Wearable motion assistance systems, especially those dedicated to lower limbs are highly promising for ambulant patients with neuromuscular disorders (e.g. FSHD, LGMD, SIBM or PD). In these conditions, the prevalence of lower-limb muscle weakness, especially in proximal muscle groups (i.e. providing strength/torque to knees) is very high. In 2014, Bouyer et al. introduced a powered knee exoskeleton, a wearable device designed to increase movement capacity during different task (6MWT, TUG, Stair Test…). This dermoskeleton has been created to enhance and augment mobility through biomechanical assistance using motorized orthopedic supports controlled by computers. This device is mainly based on the interaction of three components: 1) a network to sense the biomechanical characteristics of a specific user; 2) software for movement recognition to characterize gait phases and movement status; and 3) software to control the dermoskeleton joint mechanism to optimize biomechanical assistance. McLeod et al. demonstrated that the KeeogoTM increased performance on the 30STS, SCT and improved motor control, postural control and movement kinetics during the STS task in a chronic stroke survivor with significant hemiparesis. Recently, McGibbon et al. proved that the KeeogoTM is able to deliver an exercise-mediated benefit to individuals with MS that improved their unassisted gait endurance and stair climbing ability. However, the effect of the device on movement parameters and user perception must be specifically investigated, especially considering neuromuscular patients' specificities. We believe that ambulant patients with such disorders may highly benefit from a system that provides mobility assistance like the KeeogoTM. The use of such a device has the potential to shift the loss of ambulation and/or transfer abilities to an higher age and might mitigate disease progression as well as the occurrence of complications. Whether the KeeogoTM may be safe, usable, and efficient in ambulant patients with neuromuscular disorders, remain to be specifically investigated.

• Study Type: Interventional
• Enrollment (Actual): 50
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Paris, France, 75013
    • Institute of Myology

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 70 years (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

Description

Inclusion Criteria:

• 18 years and < 70 years of age
• Height between 1.50 m and 1.95 m
• Weight between 45 kg and 110 kg
• Abdominal perimeter < 125 cm
• Written informed consent
• Affiliate or beneficiary of a social security scheme
• Able to comply with all protocol requirements

• Confirmed diagnosis of a pathology belonging to one of the following family*:

  • Primary disorders of muscles

    • Muscular dystrophy
    • Congenital myopathies
    • Idiopathic inflammatory myopathy
    • Mitochondrial myopathies

  • Metabolic disorders

    • Inborn errors of metabolism
• Glycogen storage disease

• Functional capacities:

  • Able to stand up from a chair with armrest without other supports at least 3 times and at most 15 times in 30 seconds.
  • Report the ability to walk without the assistance of a person at least 2min
  • The use of traditional orthoses and walking aids will be accepted excepted knee orthoses and walkers (e.g. canes/crutches, ankle foot orthosis).

Exclusion Criteria:

• Unable to participate in the study
• Inability to comply with protocol requirements
• Guardianship/trusteeship
• Pregnant or nursing women
• Unstable Cardiomyopathy
• Symptomatic orthostatic hypotension
• Medical history of osteoporotic fracture
• Balance disorder with extra neuromuscular causes
• Recent trauma (fall, accident, ...)
• Unstable Cardiomyopathy
• Severe respiratory insufficiency

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Other
• Allocation: Non-Randomized
• Interventional Model: Parallel Assignment
• Masking: None (Open Label)

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Lower-limb powered dermoskeleton; Patients and healthy subjects will use a lower-limb powered dermoskeleton to perform different standardised tasks	Device: Lower-limb powered dermoskeleton; Patients and healthy subjects will wear the movement assistance device to perform different standardised physical evaluations.
No Intervention: No assistance device; Patients and healthy subjects will perform different standardised tasks without lower-limb powered dermoskeleton

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Absence of adverse effect attributable to the use of the device during task performed within a lower-limb powered dermoskeleton	Through study completion, on average 4 weeks

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Variation in the 2 Minutes Walking Test distance (express in meters) when performed using versus not using the device		Visit 2 and 3, on average 2 weeks
Variation in the 10 Meters Walking Test performance (express in seconds) when performed using versus not using the device		Visit 2 and 3, on average 2 weeks
Difference of performance with and without the device during 30 Sit To Stand		Visit 2 and 3, on average 2 weeks
Difference of performance with and without the device during simple Sit To Stand		Visit 2 and 3, on average 2 weeks
Difference of performance with and without the device during the squating test		Visit 2 and 3, on average 2 weeks
Difference of performance with and without the device during Time Up & Go test		Visit 2 and 3, on average 2 weeks
Difference of performance with and without the device during stairs climbing test		Visit 2 and 3, on average 2 weeks
Variation in spatiotemporal gait parameters during the 2 Minutes Walking Test with and without the device using 3D accelerometers' system	Spatiotemporal gait parameters: Cadence, Gait Speed, Step Duration, Stride Length, Cadence Asymmetry, Gait Speed Asymmetry, Stance Asymmetry, Stride Length Asymmetry	Visit 2 and 3, on average 2 weeks
Variation in spatiotemporal gait parameters during the 10 Meters Walking Test with and without the device using 3D accelerometers' system	Spatiotemporal gait parameters: Cadence, Gait Speed, Step Duration, Stride Length, Cadence Asymmetry, Gait Speed Asymmetry, Stance Asymmetry, Stride Length Asymmetry	Visit 2 and 3, on average 2 weeks
Variation in kinematics parameters during 30 Sit To Stand with and without the device using 3D accelerometers' system	Kinematics parameters: Gait Joint Angles, Stick Plot Visualization - Sagital plane, Hip-Knee & Knee-Ankle Plots	Visit 2 and 3, on average 2 weeks
Variation in kinematics parameters during unique Sit To Stand with and without the device using 3D accelerometers' system	Kinematics parameters: Gait Joint Angles, Stick Plot Visualization - Sagital plane, Hip-Knee & Knee-Ankle Plots	Visit 2 and 3, on average 2 weeks
Variation in kinematics parameters during Time Up & Go evaluation with and without the device using 3D accelerometers' system	Kinematics parameters: Gait Joint Angles, Stick Plot Visualization - Sagital plane, Hip-Knee & Knee-Ankle Plots	Visit 2 and 3, on average 2 weeks
Modification of lower limb muscle recruitment measured by surface EMG with and without the device		Visit 2 and 3, on average 2 weeks
Variation in postural stability measured by force platform with and without the device	Mean Velocity, RMS Sway	Visit 2 and 3, on average 2 weeks
Modification in scoring of the device measured by the Modified Nordic Questionnaire		Through study completion, on average 4 weeks
Positive scoring of device efficiency measured by the System usability scale		Through study completion, on average 4 weeks
Variation in kinematics parameters during Stairs Climbing Test with and without the device using 3D accelerometers' system	Kinematics parameters: Gait Joint Angles, Stick Plot Visualization - Sagital plane, Hip-Knee & Knee-Ankle Plots	Visit 2 and 3, on average 2 weeks

Collaborators and Investigators

• Sponsor: Institut de Myologie, France

Study record dates

Study Major Dates

• Study Start (Actual): December 1, 2021
• Primary Completion (Actual): April 22, 2025
• Study Completion (Actual): April 22, 2025

Study Registration Dates

• First Submitted: December 2, 2021
• First Submitted That Met QC Criteria: January 5, 2022
• First Posted (Actual): January 20, 2022

Study Record Updates

• Last Update Posted (Actual): April 16, 2026
• Last Update Submitted That Met QC Criteria: April 13, 2026
• Last Verified: April 1, 2026

More Information

Terms related to this study

• Keywords: Device; Exoskeleton; FSHD; Muscle; Myopathy; LGMD; Neuromuscular disorders; Motion assistance
• Additional Relevant MeSH Terms: Musculoskeletal Diseases; Nervous System Diseases; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Metabolic Diseases; Neurodegenerative Diseases; Heredodegenerative Disorders, Nervous System; Muscular Disorders, Atrophic; Carbohydrate Metabolism, Inborn Errors; Mitochondrial Diseases; Myotonic Disorders; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Muscular Dystrophies; Muscular Diseases; Myositis; Glycogen Storage Disease; Neuromuscular Diseases; Mitochondrial Myopathies; Myotonia Congenita
• Other Study ID Numbers: Exo-KGO1

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No