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DISORDERS OF THE MOTOR UNIT

Nicki Hawko, OTR/L


AGENDA

After lecture you will be able to do the following:

1. Describe the characteristics of motor unit disorders.


2. Discuss the clinical manifestations of motor unit disorders.
3. Discuss the impact of motor unit disorders on occupational performance.

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Motor unit with motor neuron cell body in the anterior horn of the spinal cord, the axon of the motor
neuron (which travels via spinal nerves and peripheral nerves to muscle), the neuromuscular junction, and
the muscle fibers innervated by the neuron.
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NEUROGENIC DISORDERS

Peripheral Neuropathies Peripheral Nerve Injuries

 Guillain-Barre syndrome  Axillary nerve injury


 Poliomyelitis  Brachial plexus injuries
 Post polio syndrome  long thoracic nerve injuries

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GUILLAIN BARRE
SYNDROME
• Acute inflammatory disorder
• Body’s immune system attacks the peripheral
nervous system
• Destroys myelin sheath
• The cause is unknown, but GBS often occurs
after a viral or bacterial infection
• Causes muscle weakness, loss of reflexes and
numbness or tingling (pins and needles) in
arms, legs, face and other parts of the body
• In severe cases it can cause complete
paralysis
• It stops getting worse within 4 weeks and
most people get better over the next 12
months

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GBS CHARACTERIZED BY;

• rapidly progressive ascending symmetric


weakness of bilateral extremities

• usually proceeding from distal to proximal


(feet to trunk)

• Descending paralysis with predominant


proximal muscle weakness rarely appears.

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GBS PHASES

1) Initial phase; when symptoms start


until they stop declining (4 ish weeks)
2) Plateau phase; when pt stabilizes with
no deterioration of physical status
and no evidence of recovery (can last
a few weeks)
3) Recovery phase; when person slowly
gets begins to recover and symptoms
decrease (6 months – 2 years)

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PROGNOSIS AND
RECOVERY FOR GBS

• No cure
• Some interventions decrease
severity of symptoms/accelerate
recovery
• Immunomodulatory therapy (IVIg)
• Plasmapheresis (decreases acute
phase)

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Start when medically stable
OT ROLE WITH GBS
Create occupational profile

ROM (start with gentle PROM then work up to AROM)

Manual muscle testing

Coordination

Self-care

Consider emotional and psychosocial factors

Pay attention to joint protection and fatigue (don’t irritate inflamed nerves)

Energy conservation

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POLIOMYELITIS

• Highly contagious viral disease


• Enters through mouth through fecal oral route
• Poor handwashing
• Enters in throat, then into blood stream
• Invasion of the CNS by circulating poliovirus via the
blood–brain barrier (BBB)
• Replicates in neurons, especially in motor neurons,
inducing the cell death that causes paralytic poliomyelitis
• Paralysis results in less than 1%

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TYPES OF Spinal polio
PARALYTIC POLIO • Most common
• Asymmetric flaccid paralysis
• Mostly in legs
Bulbar polio
• Least common
• Infects cranial nerves
• Muscle weakness in oral and facial musculature
Bulbospinal polio
• Combination of spinal and bulbar

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 After 15-40 years, some people with polio have new
symptoms
POST POLIO
SYNDROME (PPS)  Muscle pain
 Exacerbation of existing weakness
 New paralysis

 Deterioration of remaining motor neurons


 Progresses slowly
 Can have intermittent stability
 Worse if polio was severe
 Fatigue is the most debilitating

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FUNCTIONAL Increased difficulty with ADLs and IADLs
IMPLICATIONS OF Hard to walk/do stairs
POLIO AND POST
POLIO Hard to transfer
Hard to do home management tasks
Difficulty driving
Difficulty eating and swallowing
Problems with bowel and bladder
Decreased QOL
Psychological and emotional challenges
Fatigue and pain

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PERIPHERAL NERVE INJURIES

Axillary Nerve Injury Brachial Plexus Nerve Injury Long Thoracic Nerve Injury
 C5-C6 spinal nerves  Nerves C5-T1  C5-C7 nerve roots
 From upper trunk of brachial  Innervates serratus anterior
 Fibers combine and form
plexus for scapular abduction and
trunks and cords, complex
 Motor branch innervates pattern upward rotation.
deltoid and teres minor
 Not a common injury
 Most commonly injured nerve  Torn completely or partially
in shoulder  Injured by carrying heavy bag
 Sensory, motor and pain on shoulder, blow to neck
 Damage from dislocation, impairments
compression, trauma  Winging of scapula
 Injury is typically unilateral and
 Limits shoulder flexion/  Hard to reach overhead
abduction/ extension/ lateral occur during birth (stuck in
rotation canal)

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PERIPHERAL NERVE INJURY TREATMENT IDEAS

The principal goals of therapy are to:


 Maintain integrity of the joints during the recovery process.
 To achieve maximal muscle use at a tolerable pain level as the nerve is recovering.
 Educate in safety and protection due to sensory loss.
 Maintain strength in unaffected muscles.
 Assist in returning to productive activities

Interventions might include:


 Range of motion exercises and stretching
 Splinting
 Joint compression and weight bearing to facilitate muscle contraction
 Bilateral motor planning activities
 Facilitating optimal alignment in the shoulder and scapula to promote smooth movement in all directions
 Aquatic therapy when indicated

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NEUROMUSCULAR DISORDERS

Myesthenia Gravis Muscular Dystrophy


 (Neuromuscular junction)  (myopathic disorders)

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BREAK OUT GROUPS

 Create 3 slides for you assigned condition:


 Slide 1: what is it
 Slide 2: how does it present/look, how does it progress
 Slide 3: role of OT
 Conditions:
 Myasthenia Gravis
 Muscular dystrophy:
 Spinal muscular atrophy
 Limb girdle muscular dystrophy
 Myotonic muscular dystrophy
 Duchenne muscular dystrophy
 Becker muscular dystrophy
 Freidrich’s ataxia
 Fascioscapulohumeral muscular dystrophy

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THANK YOU

Presenter name
Email address
Website

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