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10 - Rehabilitation > 10-15 NM Disorders: MUscular Dystrophies, Myopathy, Spinal Muscular Atrophy, Charcot-Marie-Tooth > Flashcards

10-15 NM Disorders: MUscular Dystrophies, Myopathy, Spinal Muscular Atrophy, Charcot-Marie-Tooth Flashcards

1
Q

NM Disorders: General

A
  • Common feature: muscle weakness in muscle itself or anterior horn
  • Genetic or acquired
  • Affects any part of motor activity
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2
Q

Muscular Dystrophies

A
  • Group of muscle diseases that are genetically determined
  • No significant change in CNS (motor/sensory tracts undamaged
  • Genetic
  • Progressive degenerative disorders
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3
Q

Duchenne Muscular Dystrophy (DMD)

A
  • Psuedo Hypertrophic Muscular Dystrophy, or Progressive Muscular Dystrophy
  • Only affects males
  • Muscle cell necrosis: Unknown cause, but lack of dystrophin causes muscles to be susceptible to tears
  • Progressive weakness
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4
Q

Diagnosis of DMD (biopsy)

A
  • Degenerating fibers
  • Regenerating fibers
  • Inflammatory by-products
  • Adipose cells in connective tissue
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5
Q

Dystrophin

A
  • Protein that gives stability to muscle membrane
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6
Q

Onset of DMD

A
  • Gradual insidious onset
  • Symptoms begin between 2-5 years old
  • Reluctance to walk/run
  • Falling
  • Difficulty getting up from floor
  • Toe walking
  • Clumsiness
  • Muscle size appears to increase
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7
Q

Pseudo-hypertrophy

A
  • Deposition of adipose cells in connective tissue, including muscle
  • Muscle look strong, big, firm, but not
    Common muscles: gastrocnemius (most notable), infraspinatus, deltoid
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8
Q

DMD Muscle Weakness progression

A
  • Proximal to distal: Proximal muscles weaker earlier and progress faster
  • Exaggerated lumbar lordosis
  • Waddling Gait (Shortened ITB, broad BOS during amb, glute max weakness)
  • Gower Sign (Hallmark sign): “Climbing up legs”
  • Tightness/contractures (ITB, Hip/knee flexion, PF, Foot inversion)
  • Loss of ROM (contractures, more time sitting)
  • Perform functional activities more slowly
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9
Q

DMD Degenerative Milestones

A
  • 2-5 yo: Symptoms begin
  • 9-10 yo: Loss of unassisted ambulation
  • 12-13 yo: Complete loss of ambulation
  • 6-7 yo: Climbing stairs
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10
Q

DMD Body Systems

A
  • Scoliosis: back muscle weakness, more sitting, noticed after 11 yo; positional to fixed
  • Respiratory: Muscle weakness, atrophy, ineffective cough can lead to increased infections; too much CO2 = excessive fatigue, daytime sleepiness, headaches, sleep disturbances
  • GI: Muscle involvement, constipation. Pseudo-obstruction can cause sudden vomiting, ab pain, distention
  • Cardiac: Cardio myopathy, Arrhythmias - due to dystrophin deficiency, CHF
  • Cognition: Intellectual impairment, emotional disturbance, not progressive, not related to level of disease, may hinder development
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11
Q

DMD Medical Interventions

A
  • Spinal fixation for scoliosis
  • Scoliosis greater than 30˚
  • Especially for pt restricted to w/c
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12
Q

DMD PT Interventions

A
  • Treat symptom, not disease
  • Weakness
  • AROM/PROM
  • Locomotion
  • Functional abilities and ADLs
  • Pulmonary function
  • Emotional trauma: pt and family
  • Scoliosis
  • Pain
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13
Q

DMD PT Goals

A
  • Prevent deformity
  • Prolioing functional capacity
  • Improve pulmonary function
  • Facilitate development of family support/others
  • Control pain
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14
Q

DMD ROM and Preventing Deformity

A
  • Stretching
  • NIght splints
  • Develop scoliosis with convexity toward dominant extremity
  • Post-op: promote upright and walking when cleared
  • Bracing
  • Serial casting
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15
Q

DMD Srengthening

A
  • Allow child to self-limit and rest to prevent fatigue
  • Strengthen early
  • Endurance exercises (Swimming)
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16
Q

DMD Locomotion

A
  • Ambulation: Improve stability (stride length, BOS), bracing
  • W/C: When amb difficult, falls increase, easily fatigue
  • Consider power w/c, scooters
17
Q

DMD ADLs

A
  • Can consult OT for ADLs

- Improve function, assess and re-assess

18
Q

DMD Respiratory

A
  • Best in upright position
  • Shallow breathing (Test with pulmonary function test)
  • Weakness of diaphragm, trunk, abdominal muscles
  • PT interventions: Encourage deep breathing, bronchial drainage, chest percussion, assisted cough
19
Q

DMD Pain Management

A
  • Minimized with good PT interventions (stretching, positioning)
  • Results from impaired ROM 2˚ contractures
  • Incorporate positioning techniques to minimize pain
20
Q

Myotonic Dystrophy (MTD)

A
  • Increased muscular irritability and contractility
  • Decreased power of relaxation
  • Chromosome 19
  • Symptoms first noticed in adolescence
21
Q

MTD General

A
  • Myotonia: Delay in muscle relaxation time

- Muscle weakness, then stiffness

22
Q

MTD Muscular PRogression

A
  • Distal to proximal; Distal weakness and wasting first
  • Wasting and weakness of SCM
  • Temporal and masseter muscle wasting = long, thin face
23
Q

MTD Characteristics

A
  • Long, thin face
  • Frontal balding
  • Muscular hypoplasia (incomplete development)
  • Mental retardation
  • Cataracts
  • Numerous endocrine problems
  • Decreased respiratory drive
  • High neonatal mortality
24
Q

MTD Intervention

A
  • Reduce distal wasting
  • Reduce weakness
  • Control spinal deformities
25
Q

Limb-Girdle Muscular Dystrophy (LGMD)

A
  • Group of progressive muscular dystrophies
  • Affect proximal muscles (to distal)
  • Onset is childhood to adulthood
26
Q

LGMD Intervention

A
  • Maintain muscle length: ROM, anticipate dev of contractures, dynamic/static resting splints
  • Muscle endurance: Avoid eccentric and strengthening exercises; swimming is considered
27
Q

Congenital Muscular Dystrophy

A
  • Fukuyama CMD: Kid walk by childhood; die from resp failure

- Walker-Warburg Syndrome: More severe, child dies by age 3

28
Q

Congenital Myopathy

A
  • Muscle weakness and atrophy
  • Apparent at birth or sx onset in adulthood
  • Limits locomotion, respiratory capacity, may have fracture at birth and/or “arthyrogryposis”
29
Q

Charcot-Marie-Tooth (CMT)

A
  • Slow progressive neuropathy
  • Affects distal musculature of feet, lower legs, hands, forearms
  • Affects peripheral nerves
  • Sensory loss, weakness, wasting away
30
Q

CMT Presentation

A
  • Foot drop
  • Weakness in hands and arms
  • Contractures in feet
  • Contractures of long finger flexors
  • Distal decreased sensation to heat, touch, pain
31
Q

CMT Intervention

A
  • Strength: Resistance training
  • ROM: Stretching, night splints, serial casting, surgical intervention to fix deformities
  • Gait: Orthotics, Bracing
  • Function

10 - Rehabilitation (25 decks)

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