Use of Bioness L300 Functional

Electrical Stimulation Orthoses

in the Management of

a patient with PLS

Linda Cates, MS, PT, NCS

Duke ALS Team

Duke University Health System

Primary Lateral SclerosisAffects neurons in cerebral cortex/corticospinal tracts

Results in upper motor neuron signs

Spasticity, hyperreflexia, clonus

Usually affects Legs followed by trunk, arms/hands and later bulbar regions

Symptoms

Muscle stiffness & spasticity

Weakness

Clumsiness/slowness of movement

Balance problems

Speech and swallowing (www.ninds.nih.gov)

Primary Lateral Sclerosis

Males > females affected

Onset: 40 60 years of age

Slow progression

Not believed to be hereditary

Diagnosis of exclusion

ALS

Hereditary spastic paraparesis

Usually diagnosed over 3-4 years

(www.ninds.nih.gov)

Patient: JT symptoms onset: ~ 1999 (~ age 38)

Family history of CMT, LE spasticity

Initial symptom: pseudobulbar affect: crying:

~ 2000: reduced lower extremity limb clearance

10/2002 10/2007: followed by Duke Neurology: probable dx: Hereditary Spastic Paraparesis (HSP)

Progression of LE stiffness/gait changes/falls

2002 Fall: fracture of right radial head s/p ORIF

2003: slurring of speech/muscle spasms at night

2005/2006: 2 falls each with rib fractures

2007: progression of speech difficulties, falls, muscle spasms

Followed in Duke ALS Clinic: 1/2009 -> Present2009: significant pseudobulbar affect, UMN in bulbar,

cervical and lumbosacral segments, significant dysarthria, falls.

Family history: Genetic testing normal

Dx: probable HSP but PLS not ruled out

11/2011: on Disability, Qualified for BIPAP: FVC 68% MIP -40

Underwent additional genetic testing for HSP: negative

11/2013: Diagnosis changed to PLS

Medical Intervention ~2003: Valium (spasticity)

2004: Valium & Baclofen (spasticity)

2005: Valium (spasticity)

1/2009: Amitriptyline /discussion of baclofen pump

Pseudobulbar affect / spasticity (respectively)

11/2009: Keppra (muscle cramps)

7/2011: Amitriptyline Nuedexta (pseudobulbar affect)

11/2011: Valium Klonopin (spasticity)

10/2012: Baclofen pump implanted (spasticity)

2012 present: Baclofen Pump

Improved speech, overtime not as effective for Lower extremities

Physical Therapy PresentationStiff legged gait with poor limb clearance in swing right > left. Increased lateral sway

Excessive LE extensor tone/spasticity

Weak hip flexors, extensors and abductors (overtime: 2+/5)

Falls:

Rib fractures 2005, 2006

elbow fx 2002

AC joint injury

CMC ligament tear left hand 2009

evidence of C5-C6 compression fx on MRI following a fall

Continued frequency of falls:at times 1 x / week / 2013 10 falls reported in past year

Orthotic intervention/assistive

device recommendations

Posterior leaf (flexible) braces

LE extensor tone/spasticity

Limb clearance in swing

Assistive devices:

Stretches

Warm water aquatic therapy

Use of recumbent bike

Bioness L300 Functional Electrical Stimulation

Orthoses (NESS L300 Foot System)

wireless, software driven system designed

for drop foot following an UMN injury or

disease

Provides low level electrical stimulation

to the common peroneal nerve

The wireless Intelli-Sense Gait sensor

detects “heel on” and “heel off”

positions

communicates with the microprocessor

to adjust stimulation for level, uneven

terrain and changes in elevation

Result was Improved Limb Clearance in

Swing Phase of Gait. How did it work?

Breaking up influence of extensor tone throughout limbs by

providing electrically stimulated dorsiflexion at appropriate

parts of gait cycle

Terminal stance “heel off” to initial contact “heel on”

Allows hip flexors to fire and knees to passively flex with

less resistance due to the reduction in overall LE extensor

tone

(Bioness specific) In stance: Unit can also be programmed to

stimulate anterior tibialis to provide tibial translation and

reduce any knee recurvatum

JT without bioness

JT walking with Bioness: Anterior view

JT walking with Bioness: Lateral view

JT Bioness posterior view

Functional Outcome Measures

Gait Velocity

Without Bioness: 0.73m/sec

(0.6-> 0.8 m/sec = household ambulation)

With Bioness: 1.0m/sec

(0.8 -> 1.0 m/sec = limited community

ambulator)

1.1 m/second = norm for age.

Functional Outcome Measure

Timed Up and Go (TUG)

Without Bioness: 22.51 seconds

With Bioness: 14.01 seconds

<12 seconds = WNLs

>13.5 seconds = increased risk of falls

Functional Outcome Measure

6 minute walk testWithout Bioness: 924 feet

With Bioness: 1223 feet, 1234 feet

(on 2 different days)

> 1000 feet = community ambulator

Patient was age 52 at time of test

Norm for person 60-69 years = 1876 feet.

✔ 300 feet better with devices!!! More Efficient.

Letter of Medical Necessity

Justification for Bioness L300Well documented history

Including falls / injuries

Medical management (medicines, baclofen

pump, etc)

Physical therapy interventions (stretches,

exercise, traditional braces, assistive devices)

History of a compliant patient

Functional Outcome Measures with meaningful

change

Information about Bioness device including FDA

approval and Mechanism of how it helps patient

LMN Alone was not enough!

It Takes a Team

Patient who was willing to be his own

advocate

Go between Insurance company and

Equipment company/Bioness

Neurologist willing to speak to medical

doctor on Insurance board

Take Home Message

Bilateral Bioness L300 devices were effective in reducing spasticity during ambulation in a person with PLS.

Improved quality of life and function

Reduced risk of falls

Remaining issues

Expensive devices

Limited Insurance Coverage

Limited trials in this population