Clinical trial NCT05199246: Assessment of Safety and Acute Effects of a Lower-limb Powered Dermoskeleton in Patients With Neuromuscular Disorders
Official title: Assessment of Safety and Acute Effects of a Lower-limb Powered Dermoskeleton in Patients With Neuromuscular Disorders
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: NA
Conditions: Muscular Dystrophies
Congenital Myopathy
Idiopathic Inflammatory Myopathies
Mitochondrial Myopathies
Glycogen Storage Disease
Keywords: Neuromuscular disorders
Myopathy
Muscle
Exoskeleton
Device
Motion assistance
FSHD
LGMD
Lead sponsor: Institut de Myologie, France
Sponsor class: OTHER
First posted: 2022-01-20
Enrollment: 52
Sex: ALL
Minimum age (years): 18
Maximum age (years): 70
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Primary purpose: OTHER

Brief summary: 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.

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.

Eligibility criteria: 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

Primary outcomes:
measure: Absence of adverse effect attributable to the use of the device during task performed within a lower-limb powered dermoskeleton
timeFrame: Through study completion, on average 4 weeks
Secondary outcomes:
measure: Variation in the 2 Minutes Walking Test distance (express in meters) when performed using versus not using the device
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in the 10 Meters Walking Test performance (express in seconds) when performed using versus not using the device
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Difference of performance with and without the device during 30 Sit To Stand
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Difference of performance with and without the device during simple Sit To Stand
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Difference of performance with and without the device during the squating test
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Difference of performance with and without the device during Time Up & Go test
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Difference of performance with and without the device during stairs climbing test
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in spatiotemporal gait parameters during the 2 Minutes Walking Test with and without the device using 3D accelerometers' system
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in spatiotemporal gait parameters during the 10 Meters Walking Test with and without the device using 3D accelerometers' system
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in kinematics parameters during 30 Sit To Stand with and without the device using 3D accelerometers' system
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in kinematics parameters during unique Sit To Stand with and without the device using 3D accelerometers' system
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in kinematics parameters during Time Up & Go evaluation with and without the device using 3D accelerometers' system
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in kinematics parameters during Stairs Climbing Test with and without the device using 3D accelerometers' system
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Modification of lower limb muscle recruitment measured by surface EMG with and without the device
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Variation in postural stability measured by force platform with and without the device
timeFrame: Visit 2 and 3, on average 2 weeks
measure: Modification in scoring of the device measured by the Modified Nordic Questionnaire
timeFrame: Through study completion, on average 4 weeks
measure: Positive scoring of device efficiency measured by the System usability scale
timeFrame: Through study completion, on average 4 weeks
Other outcomes:
Locations:
city: Paris
country: France
facility: Institute of Myology
Study officials:
References: