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S7- Special Populations > Week 5- Miscellaneous Diagnoses > Flashcards

Week 5- Miscellaneous Diagnoses Flashcards

1
Q

PART 1

A

PART 1

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2
Q

Neuromuscular Diseases:

  • Includes disorders of the _________, _________, and _______.
  • No cure does not mean no treatment!
  • What are the roles of the PT?
A
  • motor neuron, neuromuscular junction, muscle

- Assist of identification, treatment of impairments, activity limitations, and participation restrictions

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3
Q

What is Muscular Dystrophy?

A
  • Group of hereditary myopathies.

- Progressive muscle weakness, deterioration, destruction, and regeneration of muscle fibers

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4
Q

In Muscular Dystrophy, muscle fibers are gradually replaced by ________ and ______ tissues.

A

fibrous and fatty tissues

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5
Q

What are (5) different types of Muscular Dystrophy?

A
  • Becker
  • Congenital
  • Congenital/myotonic
  • Child onset facioscaphum
  • Emery-Dreifus
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11
Q

Duchenne’s Muscular Dystrophy (DMD):

  • One of the most ________ and _________.
  • Death usually due to __________ and ___________ insufficiency.
  • Many are surviving into 30s.
  • Incidence between 1-3500 ______ births.
A
  • prevalent and disabling
  • respiratory and cardiorespiratory
  • males
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12
Q

Duchenne’s Muscular Dystrophy Etiology (DMD):

  • ____-linked _________ genetic disorder of X chromosome.
  • Results in a disorder of encoding __________ and __________ associated proteins (DAP).
  • Dystrophin acts as an anchor in intracellular lattice to enhance _______ strength.
  • Dystrophin absent from muscle tissue-fragility in muscle membrane stability during contraction and relative muscle hypoxia as a result of an aberrant vascular response to exercise.
  • Early breakdown of muscle fiber plasma membrane.
  • Creatine kinase (CK) ______ early in disease.
  • Muscle biopsy shows degeneration with loss of fiber, variation in fiber size, and a proliferation of CT and adipose tissue.
A
  • Sex-linked recessive genetic disorder
  • dystrophin and dystrophin associated proteins (DAP)
  • tensile strength
  • CK elevated
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13
Q

Duchenne’s Muscular Dystrophy (DMD) Clinical Presentation:

  • _________, may be misdiagnosed.
  • What are the early symptoms?
  • What is Gower’s sign?
  • Intellectual and emotional impairment.
A
  • insidious
  • Early symptoms: reluctance to walk/run at appropriate ages, falling, stair difficulty, toe walking, clumsiness, pseudohypertrophy
  • Gower’s Sign = initial weakness of neck and trunk flexors, hip extensors, interscapular muscles
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14
Q

Duchenne’s Muscular Dystrophy (DMD) S/Sx:

  • ________ muscles tend to be weaker early in disease.
  • Early weakness in _____ and _____ extensors.
  • Exaggerated lordosis, wide BOS, waddling gait, IT band contractures, heel cord contractures as progression
  • Loss of unassisted ambulation at ages __-__.
A
  • proximal
  • hip and knee extensors
  • 9-10
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15
Q

What are some surgical management techniques of DMD deformity?

A
  • Muscle release (achilles, fasciotomy of TFL and IT bands)

- Scoliosis stabilization

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16
Q

What are some things done during PT Eval of DMD?

A
  • History with family and child concerns
  • Aerobic capacity and endurance – pulmonary function testing
  • Assistive/adaptive devices
  • Community/work integration
  • Environmental barriers
  • Gait, locomotion, and balance
  • Integumentary status (when using orthotics, wheelchairs, adaptive equipment)
  • Muscle performance – MMT, dynamometer, strain gauge devices
  • Neuromotor development
  • Orthotic, protective, and supportive devices
  • Posture
  • ROM
  • Self-care and home management
  • Ventilation/respiration
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17
Q

Evidence Based Tests and Measures for DMD. (5)

A
  • Northstar Ambulatory Assessment
  • The Performance of Upper limb for Duchenne
  • Brook Scale
  • Vignos Scale-Box 12.2
  • Egen Klassifikation Scale
18
Q

DMD Prognosis:

  • Timed ________ activities closely related to muscle strength and predictive loss of ambulation.
  • 10m walk/run >__s and inability to __________ predict loss of ambulation within __ years.
  • 10m walk time >__s predicts loss of ambulation within __ years.
A
  • timed functional activities
  • > 9s and inability to rise from floor, 2 years
  • > 12s, 1 year
19
Q

What are the roles of the PT in DMD? (5)

A
  • Early diagnosis
  • Education, referral, support for family
  • Prolong function, prevent contractures and deformities, adapt equipment, encourage peer/community interaction
  • PAIN control (many have spasms and pain), massage and gentle stretching appear to help
  • Weight control, sleep/respiratory concerns, B&B concerns
20
Q

PART 2: DMD PT INTERVENTIONS

A

PART 2: DMD PT INTERVENTIONS

21
Q

DMD PT Intervention - Infancy (Preschool Age):

  • May see _______ related to weakness.
  • Mild tightness of ________ and ______.
  • Family support/education.
  • Consider daily _____ and night _________.
A
  • DD (developmental delay)
  • gastroc and TFL
  • daily ROM and night splinting
22
Q

DMD PT Intervention - Early School Age Period:

  • Limitations in activity _____ apparent - clumsiness, falling, difficulty with stairs, rise to stand, running
  • _______ deviations - increased BOS, compensated Trendelenburg, toe walking, lordosis with shoulder retraction, lack of arm swing
A
  • more apparent

- gait deviations

23
Q

What are the goals of PT intervention with Early School Age Period? (4)

A
  • Family support/education.
  • Obtain baseline data on ROM/MMT.
  • Monitor progression.
  • Maintain flexibility (especially at ankle).
24
Q

DMD Early School Age Period (STRENGTH):

  • Strength and exercise is _________.
  • Widely accepted that ________ exercise and __________ are detrimental.
  • Graded resisted exercise controversial.
  • What are the key muscles to consider?
  • Cycling, swimming, standing 2-3 hours daily.
A
  • controversial
  • eccentric and immobilization
  • abdominal, hip extensor and abductor, knee extensors
25
Q

DMD Early School Age Period (ROM):

  • Can we prevent contractures?
  • What are some key LE muscles to stretch? (3) Include stretching recommendation.
  • ____________ in combination with ________ effective and can prolong ambulation.
A
  • May not be able to prevent contractures, but can slow their development.
  • gastric, hams, TFL (1-2x/day, 10reps, 30-60s holds)
  • night splints in combination with daily stretching
26
Q

DMD Early School Age Period (RESPIRATORY):

  • What do we want to measure in regards to respiratory function?
  • Breathing exercises and inspiratory muscle training
A

-RR, chest wall excursion, coughing, secretion clearing, spirometry

27
Q

DMD Early School Age Period (FALL RISK):

  • As weakness progresses, what do we need to consider?
  • Risk of falls if ambulation continues past when it is feasible.
  • Risk for ________ increases once child is nonambulatory.
A
  • Consider other mobility options (WC, scooters, electric WC)
  • scoliosis
28
Q

DMD PT Intervention - Adolescent Period:

  • Time of considerable deterioration in function leading to what?
  • What are some UE considerations at this stage?
A
  • loss of walking, reliance of powered mobility
  • encourage participation in ADLs, adaptive equipment for self feeding, UE ergometry program, focus on maintenance of muscles for transfers
29
Q

DMD Adolescent Period (ROM):

  • Do we continue with stretching?
  • What muscles may be added to stretching?
A
  • Yes, gentle stretching continued

- hip flexors, long finger flexors, shoulder, elbow, forearm and wrist

30
Q

DMD Adolescent Period (RESPIRATORY):

  • Position of ______ is crucial.
  • What position is best to prevent scoliosis?
A
  • spine

- neutral to slightly extended (loads facet joints to prevent rotation/lateral flexion)

31
Q

DMD Adolescent Period (AMBULATION):

  • Continuation of standing/walking.
  • ________ strongly encouraged.
  • Bracing and therapeutic walking may be based on what things?
  • _________ correction may be needed to prolong ambulation.
A
  • standers
  • obesity, residual muscle, strength, absence of severe contractures, timely application of braces, motivation of family and child
  • surgical correction
32
Q

DMD Adolescent Period (FUNCTION):

  • Functional mobility/equipment
  • Stand pivot replaced by _______.
  • Sliding board inappropriate.
  • Toilet/shower equipment (consider _______ to decrease need for transfers).
  • Powered mobility
  • _____ can help.
A
  • lifts
  • urinal
  • MDA
33
Q

DMD PT Intervention - Adulthood Period:

  • After age ____, FVC and PEF decline.
  • What are 2 contributing factors to death?
  • Assisted ventilation can prolong life.
  • Breathing exercises, postural drainage.
A
  • 18

- respiratory failure and infection

34
Q

PART 3: SMA (SPINAL MUSCULAR ATROPHY)

A

PART 3: SMA (SPINAL MUSCULAR ATROPHY)

35
Q

What are the (3) different types of SMA?

A
  • Infantile (Werdnig-Hoffman)
  • Intermediate Type II
  • Juvenile (Kugelberg-Welander)
36
Q

SMA:

  • All are autosomal _________ disorders located on chromosome 5.
  • Affect ______ horn.
  • Associated with wasting/weakness of muscles.
  • Are there sensory disturbances?
A
  • autosomal recessive
  • anterior horn
  • No sensory disturbances
37
Q

What are some ways SMA is diagnosed? (5)

A
  • Clinical Presentation
  • EMG
  • Muscle Biopsy/US
  • Genetic Testing
  • Normal Cardiac Tissue
38
Q

Infantile (Werdnig-Hoffman) SMA:

  • Almost always noted in first __ months of life.
  • Often _______ fetal movement.
  • _______ but appears alert and responsive.
  • Difficulty feeding, respiratory distress.
  • Muscle wasting with few spontaneous movements.
  • _____ decreased or absent.
  • Limited lifespan, respiratory failure.
A
  • 3 months
  • decreased
  • hypotonia
  • DTRs
39
Q

Infantile (Werdnig-Hoffman) SMA:

  • What are some secondary impairments of Infantile SMA? (4)
  • What are some PT interventions that can be utilized with these patients?
  • Limited use of ______ due to difficulty lifting head, inhibition of abdominal expansion and diaphragmatic depression.
  • Breathing may become difficult in upright as well.
A
  • scoliosis, contractures, decreased respiratory capacity, fatigability
  • ROM, feeding, positioning, respiratory care, select developmental activities, assistive technology
  • prone
40
Q

Intermediate (Type II) SMA:

  • Also affects infants, but is it more or less severe than Infantile SMA?
  • Floppy, slow to develop motor milestones.
  • Usually diagnoses __-__m of age.
  • Weak ______/_________ with muscle atrophy.
  • Few have feeding problems.
  • Fine extremity _________.
  • May or may not learn to _____.
  • Most require ______ intervention.
A
  • less severe
  • 3-6m
  • trunk/extremities
  • tremors
  • walk
  • orthotic
41
Q

What are (4) goals of Intermediate SMA?

A
  • Encourage stance
  • Prevent deformity
  • Respiratory interventions
  • Adapt play and writing tools
42
Q

Juveline (Kugelberg-Welander) SMA:

  • Diagnosed between __-__ years.
  • _____ progressive weakness with mild impairment.
  • Are proximal or distal muscles usually involved first?
A
  • 1-10 years
  • slowly progressive
  • proximal muscles

S7- Special Populations (18 decks)

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