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.

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

Muscular Dystrophies

Congenital Myopathy

Idiopathic Inflammatory Myopathies

Mitochondrial Myopathies

Glycogen Storage Disease

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.

Lower-limb powered dermoskeleton

Spatiotemporal gait parameters: Cadence, Gait Speed, Step Duration, Stride Length,
Cadence Asymmetry, Gait Speed Asymmetry, Stance Asymmetry, Stride Length Asymmetry

Kinematics parameters: Gait Joint Angles, Stick Plot Visualization - Sagital plane,
Hip-Knee & Knee-Ankle Plots

Patients and healthy subjects will use a lower-limb powered dermoskeleton to perform
different standardised tasks

Patients and healthy subjects will perform different standardised tasks without
lower-limb powered dermoskeleton

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

About this trial

Study ID

Status

Type

Phase

Placebo

Accepting

Trial Timing

What is this trial about?

What are the participation requirements?

Locations

Location

Status