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PTH 635 Peds > Exam 2 > Flashcards

Exam 2 Flashcards

torticollis, CP, hypotonia, DS, DCD, MD

1
Q

Congential muscular torticollis -CMT- (wry neck/twisted neck) - how does it happen?

A

-most common accepted = ischemia, birth trauma, and intrauterine malposition

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2
Q

Torticollis - prevalence

A
  • 3rd most common anomaly (after dislocated hip and club foot)
  • 0.3-16% of newborns
  • incidence has inc dramatically since “Back to sleep” campaign
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3
Q

“Flathead syndrome”

A
  • plagiocephaly

- ***is reported as a coexisting impairment in 80-90.1% of kids with CMT (toricollis)

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4
Q

plagiocephaly risk factors

A
  • large birth wt
  • male
  • breech position
  • multiple births
  • first delivery
  • difficult labor
  • nuchal cord
  • maternal uterine abnormalities
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5
Q

types of CMT

A
  • sternomastoid tumor
  • muscular torticollis
  • postural torticollis
  • postnatal muscular torticollis
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6
Q

sternomastoid tumor (type of CMT)

A

-discrete mass is palpable within the SCM muscle

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7
Q

muscular torticollis - (type of CMT)

A

-tightness, but no palpable mass

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8
Q

postural torticollis (type of CMT)

A

-no SCM tightness, no palpable mass

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9
Q

postnatal muscular torticollis (type of CMT)

A

-environment, plagiocephaly, positional preference, associated with other birth problems - CP, myelodysplaia, down syndrome etc.

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10
Q

torticollis - decreased neck ROM for?

A
  • ipsilateral rotation
  • contralateral LF
  • contralateral asymmetrical flex/ext

-also: unable to maintain midline alignment

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11
Q

other body impairments associated with torticollis

A
  • anterior neck (platysma, scalenes, hyoids, tongue, and facial muscles
  • trunk curvature
  • persistence of asymmetric ATNR
  • pelvic obliquities
  • shoulder elevation
  • congenital scoliosis
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12
Q

torticollis impact on sensory system

A
  • vision
  • vestibular
  • somatosensation to regulate posture
  • kinesthetic feedback

*overall systems developing asymmetrically and not experiencing normal interactions of each system as the child grows

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13
Q

torticollis - typical activity limitations

A
  • neglect of ipsilateral hand
  • asymmetrical head righting reaction
  • delayed propping-prone
  • delayed rolling over
  • limited vestibular, proprioceptive, sensorimotor
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14
Q

torticollis - impairments related to activity limitations

A
  • limited ROM lead to compensatory mvmts
  • dec neck LF leads to overuse of torso muscles
  • difficult maintaining midline in upright position
  • regaining midline head posture in vertical, prone, supine, with wt shifts, as well as with movement
  • UE wt bearing difficult on involved side
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15
Q

deformational plagiocephaly

A

most common - Left - CMT-right occipital flattening

  • can be same or opposite side to muscular torticollis at birth
  • acquired = develops in first 3 months. Always same side of preferred rotation
  • masticatory mm weaker on affected SCM
  • progression slows around 6 months due to brain growth slow and can sit more.
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16
Q

anterior fontanelle -closes when

A
  • last to close

- close around 9-18 months of age

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17
Q

posterior fontanelle - closes when

A

closes 1-2 months.

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18
Q

TIMP (test of infant motor performance)

A
  • infant 32 weeks gestation.
  • age 4-5 months post term
  • has a good part that assesses infant ability to index. control head position in a variety of spatial orientations
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19
Q

torticollis - interventions

A
  • passive neck ROM
  • AAROM
  • strengthening
  • postural control
  • caregiver education - carrying, positioning, ROM at home

-*overall goals to restore full joint and muscle ROM, prevent contractors, restore strength.

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20
Q

studies about initiating exercise before 1 year of age???

A

-PROM of neck reported as good to excellent with success 61-99%

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21
Q

cranial orthotics

A
  • DOC band
  • hanger band
  • star band

*measurements mod-severe then refer out.

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22
Q

torticollis - anticipated outcomes

A
  • full PROM of neck, trunk, extremities
  • active and symmetric head rotation in all positions
  • active midline head control
  • normal antigravity strength
  • normal head righting
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23
Q

What is Cerebral palsy?

A
  • permanent, non-progressive disorder as a result of a brain lesion within the first 2 years of life.
  • have progressive musculoskeletal problems
  • can also have: cognitive delay, behavioral issues, impaired speech hearing vision, seizures, urinary incontinence, constipation, etc
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24
Q

CP - Diplegia

A
  • both sides of body

- typically legs more affected than arms

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25
Q

CP - hemiplegia or hemiparesis

A
  • one side of body (arm and leg)
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26
Q

CP - Triplegia

A
  • 3 limbs
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27
Q

CP - quadriplegia or tetraplegia

A
  • 4 limbs
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28
Q

Spastic CP

A
  • results from involvement of motor cortex or white matter projections to and from the cortical sensorimotor areas of the brain
  • spasticity and exaggerated reflexes result in abnormal patterns of posture and movement
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29
Q

Dyskinesia CP

A
  • involvement of basal ganglia
  • atypical patterns of posture and involuntary, uncontrolled, recurring, and occasionally stereotyped movements
  • dystonic or athetosis subtypes

**uncontrolled, slow

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30
Q

Ataxic CP

A
  • cerebellar lesion
  • inability to generate normal or expected voluntary movement trajectories

**poor coordination and balance

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31
Q

Mixed CP

A

-symptoms of spastic and dyskinesia may be present

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32
Q

Prevalence of CP types

A
  • spastic hemi 30%
  • spastic diplegia 38%
  • spastic quad 5.5%
  • dystonia 9.5%
  • athetosis 5.5%
  • ataxic forms 11%
  • mixed 2%

*spastic > ataxic

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33
Q

causes of CP

A

(pre-, peri-, and postnatal)

  • hypoxic
  • ischemic
  • infections
  • congential
  • traumatic insults
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34
Q

what is the most common cause of physical disability affecting kids in developed countries?

A

-CP (2-2.5/1000 live births)

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35
Q

diagnosis CP

A
  • kid does not reach motor milestones and shows abnormal tone or qualitative differences in mvmt patterns.
  • neuro imaging
  • prenatal risk factors
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36
Q

determining prognosis in CP

A
    • sitting independently by 24 months is best predictor of ambulation with or w/o AD by age 8 (if not achieved by age 3 walking is little chance)
  • cognitive function is a strong predictors
  • 54% walk independently by age 5
  • hemiplegic and ataxic more likely to walk
  • dyskinetic and bil CP least likely
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37
Q

positive factors for CP prognosis

A
  • mild physical involvement
  • good home support
  • education
  • vocational training
  • good cognitive skills

-31% of adults live independently

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38
Q

CP - life expectancy

A
  • 2 y/o with severe CP has 40% chance to live to 20 (mild CP = 99%)
  • decline in mortality of kids with CP in past 20 years due to better management
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39
Q

modified tardieu scale

A
  • for tone
  • measure point of resistance or “catch” to a rapid velocity stretch
  • good indicator of dynamic neural tone or overactive stretch reflex
40
Q

standardized tests for infants -CP

A
  • TIMP
  • AIMS
  • Movement Assessment of Infants
41
Q

hypotonicity definition

A

-dec resistance to passive stretch

involved in DS, genetic disorders, MD, hypotonia

42
Q

Early warning signs of kids with hypotonia

A

parents notice excessive floppiness and general inactivity

43
Q

basic problems with kids with hypotonia - consequences

A
  • difficult maintaining a secure posture to interact with the environment
  • can limit cognitive development
  • inc self-esteem behavior to fill void of sensory input
44
Q

hypotonia - postural instability

A
  • sink into gravity
  • limit early random play
  • poor midline control
  • lack dynamic muscle control
  • use either total flexion or extension patterns
  • lacks graded control of all 3 phases of muscle activity (initiate, sustain, terminate)
45
Q

hypotonia - hypermobility

A
  • lack of ligament, muscle, and tissue resistance toward extreme movement ranges
  • sustained posturing in extreme joint ranges can cause reduced mobility in the opposing joint range
46
Q

hypotonia - possible deformities

A
  • hip dislocation

- pelvic torsion

47
Q

hypotonia - respiration

A
  • insufficient for sustained vocalization

- breathing is noisy d/t rib cage instability

48
Q

Down syndrome - cause

A
  • additional chromosome (47 total instead of 46)

- commonly trisomy 21 (nondisjunction 95%, translocation/mosaic 5%)

49
Q

down syndrome - incidence and life expectancy

A
  • 1 in 800-1000
  • more common in older moms giving birth

-life expectancy = 60s

50
Q

down syndrome - neuropathology

A
  • dec wt of brain
  • microcephaly or microbrachycephaly
  • secondary sulci reduced-simplicity of convoluted patterns on the brain
  • motor incoordination
  • lack of myelination between 2-6 years of age
  • up to 8% have seizure disorder
51
Q

DS - sensory deficits

A
  • visual (congenital, cataracts, myopia, farsightedness, strabismus, nystagmus)
  • heading deficits (60-80% mild to moderate loss)
  • speech impairments
52
Q

DS - cardiopulmonary

A
  • 66% born with congenital heart defects (most common AV canal defects and ventriculoseptal defects)
  • usually repaired in infancy
  • if not repaired by age 3 = high association with greater delays in motor skills
53
Q

DS - musculoskeletal linear growth deficits

A

greatest between 6-24 months of age

  • leg-length reduction
  • 10-30% reduction in metacarpal and phalangeal length
54
Q

DS - musculoskeletal - muscle variations

A
  • absent palmaris longus

- lack differentiation of distinct mm bellies for zygomaticus major/minor and levator labii superior of the face

55
Q

DS - musculoskeletal - hypotonia

A
  • found in all muscle groups
  • ***hallmark feature in kids with DS
  • major contributing factor for delays
56
Q

DS - musculoskeletal - ligamentous laxity

A
  • another hallmark feature of kids with DS

- collagen deficit

57
Q

DS - musculoskeletal - most commonly see

A
  • pes planus
  • petallar instability
  • scoliosis
  • atlantoaxial instability
  • hip subluxation
58
Q

DS - distinguishing features

A
  • small head, mouth, palate, eyes, low muscle tone
  • single deep crease on palm of hand
  • low set ears
59
Q

how does DS influence calories burned?

A

kids with DS burn 10-15% less calories

60
Q

Developmental coordination disorder (DCD) - definition

A
  • poorly defined fine and/or gross motor skills
  • not attributed to a known neurological or medical disorder
  • unknown pathologic process **
61
Q

DCD is believed to involve what areas of the brain?

A

cerebellum and basal ganglia

62
Q

diagnosing DCD

A
  • motor impairments and skills delay significant impacts child’s activities
  • adequate opportunities for experience and practice
  • no other explanations offered for impairments
63
Q

DCD primary impairments

A
  • imp vision, kinesthetics, proprioception
  • slow and awkward movements. rigid and jerky
  • inappropriate and ineffective muscle activation and sequence
  • poor motor learning
64
Q

DCD secondary impairments

A
  • tired

- frequently “off task”

65
Q

DCD activity limitations

A

-limited fine and gross motor skills

Fine: tie shoes, zippers, open food, writing

Gross: bike, swing, run, stairs, etc.

66
Q

DCD key questions to ask yourself

A
  • inc or fluctuating tone?
  • delays more global?
  • have difficulties been present from an early age?
  • are motor concerns worsening?
  • any loss of previously acquired skills?
67
Q

Developmental coordination disorder questionnaire -assessment tool

A

-parent report

68
Q

teacher reported DCD assessments

A
  • MABC-C and MABC checklist-2
  • ChAST
  • MOQ-T
69
Q

assessment tools - Peabody

A

-young children

70
Q

assessment tools - BOTMP (“BOT”)

A
  • older kids

- measures ability, but not quality of movement

71
Q

assessment tools - MABC

A
  • older kids

- *best assessment tool for DCD in spite of omitting handwriting

72
Q

DCD interventions - bottom up approach

A
  • focus on components of skills

- aimed primarily at changing body structure/function impairments

73
Q

DCD interventions - top down approach

A
  • dynamic systems theory

- emphasis on specific skills not components.

74
Q

Muscular dystrophy (MD) - info

A
  • genetic inheritance
  • progressive neuromuscular disease caused by destruction of myofibrils
  • incurable, but treatable
75
Q

MD characteristics

A
  • progressive weakness
  • atrophy
  • contractures
  • deformity and progressive disability
76
Q

Duchene (DMD) - onset, inheritance, course

A
  • onset = 1-4 years
  • inheritance = X-linked
  • course = rapidly progressive. loss of walking by 9 y/o. death in late teens.

**gower sign

77
Q

Becker (BMD) - onset, inheritance, course

A
  • onset = 5-10 yrs
  • inheritance = X-linked
  • course = slow progressive. maintain walking past early teens. life span into 3rd decade
78
Q

Congenital MD -onset, inheritance, course

A
  • onset = birth
  • inheritance = recessive
  • course = typically slow but variable. shortened life span
79
Q

Congenital myotonic MD - onset, inheritance, course

A
  • onset = birth
  • inheritance = dominant
  • course = typically slow with significant intellectual impairment
80
Q

Childhood-onset facioscapulohumeral MD -onset, inheritance, course

A
  • onset = first decade
  • inheritance = dominant/recessive
  • course = slowly progressive loss of walking in later life. variable life expectancy
81
Q

Emery-Dreifus MD - onset, inheritance, course

A
  • onset = childhood - early teens
  • inheritance = X-linked
  • course = slowly progressive with cardiac abnormality and normal life span
82
Q

MD - primary impairments

A

-insidious weakness secondary to progressive loss of myofibrils

83
Q

MD - secondary impairments

A

-contractures, postural mal-alignments (scoliosis), dec respiratory capacity, fatigability, obesity

84
Q

Duchenne’s MD cause and symptoms

A
  • 1 in 3500 births
  • cause = absence of dystrophin
  • symptoms = gen weakness and muscle wasting. (prox first)
  • calves are often enlarged**
85
Q

goal of PT with people with MD.

A
  • prolong independence
  • slow progression of complications
  • improve quality of life

*focus on mobility and prevent contractors and improve strength

86
Q

MD - to exercise or not to exercise?

A
  • exercise moderately but do not go to exhaustion

- aquatics are good. buoyancy

87
Q

Spinal muscular atrophy (SMA)

A
  • a genetic disease that causes a loss of motor neurons in spinal cord
  • caused by a deficiency of a motor neuron protein called SMN (survival motor neuron?)
  • on chromosome 5. is rare X-linked
  • onset = before birth to 6 months
  • 4 types. type 1 and 2 most prevalent
88
Q

Most common genetic cause of death in infancy?

A

SMA - spinal muscle atrophy

89
Q

SMA - symptoms

A

-generalized muscle weakness, weak cry, trouble swallowing as well as sucking, and breathing distress. cannot sit without support

90
Q

SMA - progression

A

-very rapidly

91
Q

SMA type 1

A

(infantile-onset, werdnig-hoffmann disease)

  • most severe form
  • cannot sit independently
  • between birth and 6 month old
  • 50% die before 2nd birthday
92
Q

SMA type 2

A

(intermediate SMA)

  • affect babies before 18 months
  • may be able to sit unaided or stand with support
  • shortened life span
93
Q

SMA type 3

A

(juvenile SMA, Kugelberg-Welander disease)

  • mildest form
  • between 18 months and late adolescence
  • can stand and walk independently for some time
94
Q

SMA type 4

A

(adult SMA)

  • adult form of disease
  • symptoms begin after age 35
95
Q

SMA primary impairments

A

-muscle weakness secondary to progressive loss of anterior horn cells in spinal cord

96
Q

SMA secondary impairments

A
  • Cranial nerve involvement
  • contractures
  • muscle fasciculations
  • respiratory distress
  • scoliosis
  • fatigability
97
Q

SMA - treatment

A

-focus on feeding, ROM, positioning, respiratory care and selected developmental activities

PTH 635 Peds (4 decks)

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