Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

special pops > Muscular Dystrophies and Spinal Muscular Atrophy > Flashcards

Muscular Dystrophies and Spinal Muscular Atrophy Flashcards

1
Q

PT role w/neuromuscular diseases

A
  1. assist in ID
  2. treat impairments
  3. activity limitations with ADs, orthotics, and interventions
  4. participation restrictions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Muscular Dystrophy

A

group of hereditary myopathies

  • progressive muscle weakness, deterioration, and regeneration of muscle fibers
  • muscle fibers gradually replaced by fibrous and fatty tissue
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Duchenne’s Muscular Dystrophy

A
  • one of the most prevalent/disabling
  • death usually respiratory or CV insufficiency
  • survival into 30s
  • 1 / 3500 male births
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Duchenne’s:

etiology

A
  1. recessive
  2. X chromosome abnormality
  3. results in disorder dystrophin and DAP
  4. dystrophin acts as anchor in intracellular lattice to enhance tensile strength
  5. fragility in muscle membrane during contraction and hypoxia
  6. early breakdown of muscle fiber plasma membrane
  7. CK elevated early in disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Duchenne’s: pseudoatrophy

A

tissue replaced w/fatty and connective tissue

gastroc, deltoids, quads, forearm extensors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Duchenne’s Clinical Presentation

A
  1. insidious, often misdiagnosed
  2. early reluctance to walk/run.
  3. falling
  4. difficulty w/stairs
  5. toe walking
  6. clumsiness
  7. Gower’s sign
  8. intellectual impairment/emotional
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Duchenne’s signs/symptoms

A
  1. proximal muscles weaker early
  2. early weakness in hip/knee extensors
  3. exaggerated lordosis, wide BOS, waddling gait, band contractures, heel cord contractures
  4. los of unassisted ambulation 9-10 y.o
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Duchenne’s medical intervention

A
  1. steroids (primary trx)
  2. myoblast transplant
  3. gene therapy
  4. cell based replacement
  5. creatine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Duchenne’s: steroid therapy benefits

A
  • prolongs walking by 3 years
  • improvements in isometric UE str by 60%, LE 85%
  • improved pulmonary function
  • improved cog function
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Duchenne’s: steroid therapy AE

A
  1. weight gain
  2. growth suppression
  3. cataracts
  4. osteoporosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Duchenne’s surgical management of deformity

A
  1. muscle release for Achilles
  2. fasciotomy of TFL/ITB
  3. scoliosis stabilization
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Duchenne’s evidence based tests/measures

A
  1. Northstar Ambulatory Assessment
  2. The Performance of Upper limb for Duchenne
  3. Brook Scale
  4. Vignos Scale
  5. Egen Klassifikation Scale
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Duchenne’s Prognosis

A
  • Timed functional activities closely related to muscle str and loss of ambulation
  • 10 m walk/run time > 9 sec and inability to rise from floor predicts loss of ambulation within 2 years
  • 10 m walk
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Duchenne’s:

10m walk time > 9sec and inability to rise from floor

A

predicts loss of ambulation within 2 years

consider WC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Duchenne’s:

10 m walk time > 12 sec

A

loss of ambulation within a year

order WC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Duchenne’s: PT role

A
  1. early dx
  2. education, referral, family support
  3. prolong function
  4. pain control
  5. weight control
  6. B&B concerns
  7. sleep/respiratory concerns
17
Q

Duchenne’s: prolong function

A
  • prevent contractures/deformities
  • ADs
  • encourage peer/community interaction
18
Q

Duchenne’s: Pain control

A

massage and gentle stretching appear to help w/spasms

worse in adolescence when they lose ambulation

19
Q

Duchenne’s PT: infancy-preschool

A
  • family education
  • preventative ROM
  • mild tightness=night splint + AFO + slant board
  • aerobic conditioning and functional str
20
Q

Duchenne’s PT: early school age

A

advocate for assistance and modifications to days in school.

  • clumsiness
  • difficulty w/stairs
  • gait deviations
21
Q

Duchenne’s early school age:

gait deviations

A
  • increased BOS
  • compensated Trendelenburg
  • toe walking
  • lordosis with shoulder retraction
  • lack of arm swing
22
Q

Duchenne’s early school age goals

A
  1. family support/edu
  2. obtain baseline ROM/MMT
  3. monitor progression
  4. maintain flexibility
23
Q

Duchenne’s: early school age: Strength and exercise

A

eccentric exercise and immobilization are detrimental

graded resistance controversial

24
Q

Duchenne’s key muscles

A

abs, hip ext/ABD, knee EXT

cycling, swimming, standing 2-3 hrs daily

25
Q

Duchenne’s key muscles to stretch

A

gastroc, hams, TFL

1-2/day. up to 10 reps. 30-60 sec

night splints + day stretching most effective

26
Q

Duchenne’s early school age respiratory function

A
  • measure RR, chest wall excursion, coughing, secretion clearing, spirometry
  • breathing exercises, inspiratory muscle training
27
Q

Duchenne’s adolescent intervention

A
  • encourage participation in ADLs
  • UE ergometry
  • maintain muscles for transfers
  • LE ROM
  • Respiratory function
  • standers encouraged for walking
  • bracing
  • surgery
28
Q

Duchenne’s adolescence:

why is the position of the spine crucial?

A

neutral to slightly extended best to prevent scoliosis

29
Q

Duchenne’s interventions:

Adulthood

A

After age of 18, FVC and PEF decline

  1. BPAP
  2. neg pressure ventilators
  3. suctioning
  4. breathing exercise
  5. postural drainage
30
Q

SMA I: Wernig-Hoffman

A
  • often noted 1st 3 months of life
  • often decreased fetal movement
  • hypotonia
  • difficulty feeding
  • respiratory distress
  • muscle wasting
  • DTRs decreased/absent
  • limited lifespan
31
Q

SMA type I: secondary impairments

A
  1. scoliosis
  2. contractures
  3. dec respiratory capacity
  4. fatiguability
32
Q

SMA type I: PT

A
  1. ROM
  2. feeding
  3. positioning
  4. respiratory care
  5. select developmental activities
  6. assistive tech
33
Q

SMA type I: why is there limited use of prone

A
  • difficulty lifting head

- inhibition of abdominal expansion and diaphragmatic depression

34
Q

SMA Intermediate

A
  • floppy
  • delayed motor milestones
  • diagnosed 3-6 months
  • weak trunk/extremities w/atrophy
  • fine extremity tremors
  • may or may not learn to walk
  • most need orthotics
35
Q

SMA Intermediate: PT

A
  1. encourage stance
  2. prevent deformity
  3. respiratory interventions
  4. adapt play and writing tools
36
Q

Juvenile- Kugelberg-Welander

A
  1. Diagnosed between 1 and 10
  2. Slowly progressive weakness, mild impairment
  3. Proximal muscles are usually involved first

special pops (11 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions