Exam 2 Flashcards
torticollis, CP, hypotonia, DS, DCD, MD
Congential muscular torticollis -CMT- (wry neck/twisted neck) - how does it happen?
-most common accepted = ischemia, birth trauma, and intrauterine malposition
Torticollis - prevalence
- 3rd most common anomaly (after dislocated hip and club foot)
- 0.3-16% of newborns
- incidence has inc dramatically since “Back to sleep” campaign
“Flathead syndrome”
- plagiocephaly
- ***is reported as a coexisting impairment in 80-90.1% of kids with CMT (toricollis)
plagiocephaly risk factors
- large birth wt
- male
- breech position
- multiple births
- first delivery
- difficult labor
- nuchal cord
- maternal uterine abnormalities
types of CMT
- sternomastoid tumor
- muscular torticollis
- postural torticollis
- postnatal muscular torticollis
sternomastoid tumor (type of CMT)
-discrete mass is palpable within the SCM muscle
muscular torticollis - (type of CMT)
-tightness, but no palpable mass
postural torticollis (type of CMT)
-no SCM tightness, no palpable mass
postnatal muscular torticollis (type of CMT)
-environment, plagiocephaly, positional preference, associated with other birth problems - CP, myelodysplaia, down syndrome etc.
torticollis - decreased neck ROM for?
- ipsilateral rotation
- contralateral LF
- contralateral asymmetrical flex/ext
-also: unable to maintain midline alignment
other body impairments associated with torticollis
- anterior neck (platysma, scalenes, hyoids, tongue, and facial muscles
- trunk curvature
- persistence of asymmetric ATNR
- pelvic obliquities
- shoulder elevation
- congenital scoliosis
torticollis impact on sensory system
- vision
- vestibular
- somatosensation to regulate posture
- kinesthetic feedback
*overall systems developing asymmetrically and not experiencing normal interactions of each system as the child grows
torticollis - typical activity limitations
- neglect of ipsilateral hand
- asymmetrical head righting reaction
- delayed propping-prone
- delayed rolling over
- limited vestibular, proprioceptive, sensorimotor
torticollis - impairments related to activity limitations
- 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
deformational plagiocephaly
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.
anterior fontanelle -closes when
- last to close
- close around 9-18 months of age
posterior fontanelle - closes when
closes 1-2 months.
TIMP (test of infant motor performance)
- 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
torticollis - interventions
- 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.
studies about initiating exercise before 1 year of age???
-PROM of neck reported as good to excellent with success 61-99%
cranial orthotics
- DOC band
- hanger band
- star band
*measurements mod-severe then refer out.
torticollis - anticipated outcomes
- full PROM of neck, trunk, extremities
- active and symmetric head rotation in all positions
- active midline head control
- normal antigravity strength
- normal head righting
What is Cerebral palsy?
- 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
CP - Diplegia
- both sides of body
- typically legs more affected than arms
CP - hemiplegia or hemiparesis
- one side of body (arm and leg)
CP - Triplegia
- 3 limbs
CP - quadriplegia or tetraplegia
- 4 limbs
Spastic CP
- 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
Dyskinesia CP
- involvement of basal ganglia
- atypical patterns of posture and involuntary, uncontrolled, recurring, and occasionally stereotyped movements
- dystonic or athetosis subtypes
**uncontrolled, slow
Ataxic CP
- cerebellar lesion
- inability to generate normal or expected voluntary movement trajectories
**poor coordination and balance
Mixed CP
-symptoms of spastic and dyskinesia may be present
Prevalence of CP types
- spastic hemi 30%
- spastic diplegia 38%
- spastic quad 5.5%
- dystonia 9.5%
- athetosis 5.5%
- ataxic forms 11%
- mixed 2%
*spastic > ataxic
causes of CP
(pre-, peri-, and postnatal)
- hypoxic
- ischemic
- infections
- congential
- traumatic insults
what is the most common cause of physical disability affecting kids in developed countries?
-CP (2-2.5/1000 live births)
diagnosis CP
- kid does not reach motor milestones and shows abnormal tone or qualitative differences in mvmt patterns.
- neuro imaging
- prenatal risk factors
determining prognosis in CP
- 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
positive factors for CP prognosis
- mild physical involvement
- good home support
- education
- vocational training
- good cognitive skills
-31% of adults live independently
CP - life expectancy
- 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
modified tardieu scale
- for tone
- measure point of resistance or “catch” to a rapid velocity stretch
- good indicator of dynamic neural tone or overactive stretch reflex
standardized tests for infants -CP
- TIMP
- AIMS
- Movement Assessment of Infants
hypotonicity definition
-dec resistance to passive stretch
involved in DS, genetic disorders, MD, hypotonia
Early warning signs of kids with hypotonia
parents notice excessive floppiness and general inactivity
basic problems with kids with hypotonia - consequences
- difficult maintaining a secure posture to interact with the environment
- can limit cognitive development
- inc self-esteem behavior to fill void of sensory input
hypotonia - postural instability
- 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)
hypotonia - hypermobility
- 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
hypotonia - possible deformities
- hip dislocation
- pelvic torsion
hypotonia - respiration
- insufficient for sustained vocalization
- breathing is noisy d/t rib cage instability
Down syndrome - cause
- additional chromosome (47 total instead of 46)
- commonly trisomy 21 (nondisjunction 95%, translocation/mosaic 5%)
down syndrome - incidence and life expectancy
- 1 in 800-1000
- more common in older moms giving birth
-life expectancy = 60s
down syndrome - neuropathology
- 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
DS - sensory deficits
- visual (congenital, cataracts, myopia, farsightedness, strabismus, nystagmus)
- heading deficits (60-80% mild to moderate loss)
- speech impairments
DS - cardiopulmonary
- 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
DS - musculoskeletal linear growth deficits
greatest between 6-24 months of age
- leg-length reduction
- 10-30% reduction in metacarpal and phalangeal length
DS - musculoskeletal - muscle variations
- absent palmaris longus
- lack differentiation of distinct mm bellies for zygomaticus major/minor and levator labii superior of the face
DS - musculoskeletal - hypotonia
- found in all muscle groups
- ***hallmark feature in kids with DS
- major contributing factor for delays
DS - musculoskeletal - ligamentous laxity
- another hallmark feature of kids with DS
- collagen deficit
DS - musculoskeletal - most commonly see
- pes planus
- petallar instability
- scoliosis
- atlantoaxial instability
- hip subluxation
DS - distinguishing features
- small head, mouth, palate, eyes, low muscle tone
- single deep crease on palm of hand
- low set ears
how does DS influence calories burned?
kids with DS burn 10-15% less calories
Developmental coordination disorder (DCD) - definition
- poorly defined fine and/or gross motor skills
- not attributed to a known neurological or medical disorder
- unknown pathologic process **
DCD is believed to involve what areas of the brain?
cerebellum and basal ganglia
diagnosing DCD
- motor impairments and skills delay significant impacts child’s activities
- adequate opportunities for experience and practice
- no other explanations offered for impairments
DCD primary impairments
- imp vision, kinesthetics, proprioception
- slow and awkward movements. rigid and jerky
- inappropriate and ineffective muscle activation and sequence
- poor motor learning
DCD secondary impairments
- tired
- frequently “off task”
DCD activity limitations
-limited fine and gross motor skills
Fine: tie shoes, zippers, open food, writing
Gross: bike, swing, run, stairs, etc.
DCD key questions to ask yourself
- 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?
Developmental coordination disorder questionnaire -assessment tool
-parent report
teacher reported DCD assessments
- MABC-C and MABC checklist-2
- ChAST
- MOQ-T
assessment tools - Peabody
-young children
assessment tools - BOTMP (“BOT”)
- older kids
- measures ability, but not quality of movement
assessment tools - MABC
- older kids
- *best assessment tool for DCD in spite of omitting handwriting
DCD interventions - bottom up approach
- focus on components of skills
- aimed primarily at changing body structure/function impairments
DCD interventions - top down approach
- dynamic systems theory
- emphasis on specific skills not components.
Muscular dystrophy (MD) - info
- genetic inheritance
- progressive neuromuscular disease caused by destruction of myofibrils
- incurable, but treatable
MD characteristics
- progressive weakness
- atrophy
- contractures
- deformity and progressive disability
Duchene (DMD) - onset, inheritance, course
- onset = 1-4 years
- inheritance = X-linked
- course = rapidly progressive. loss of walking by 9 y/o. death in late teens.
**gower sign
Becker (BMD) - onset, inheritance, course
- onset = 5-10 yrs
- inheritance = X-linked
- course = slow progressive. maintain walking past early teens. life span into 3rd decade
Congenital MD -onset, inheritance, course
- onset = birth
- inheritance = recessive
- course = typically slow but variable. shortened life span
Congenital myotonic MD - onset, inheritance, course
- onset = birth
- inheritance = dominant
- course = typically slow with significant intellectual impairment
Childhood-onset facioscapulohumeral MD -onset, inheritance, course
- onset = first decade
- inheritance = dominant/recessive
- course = slowly progressive loss of walking in later life. variable life expectancy
Emery-Dreifus MD - onset, inheritance, course
- onset = childhood - early teens
- inheritance = X-linked
- course = slowly progressive with cardiac abnormality and normal life span
MD - primary impairments
-insidious weakness secondary to progressive loss of myofibrils
MD - secondary impairments
-contractures, postural mal-alignments (scoliosis), dec respiratory capacity, fatigability, obesity
Duchenne’s MD cause and symptoms
- 1 in 3500 births
- cause = absence of dystrophin
- symptoms = gen weakness and muscle wasting. (prox first)
- calves are often enlarged**
goal of PT with people with MD.
- prolong independence
- slow progression of complications
- improve quality of life
*focus on mobility and prevent contractors and improve strength
MD - to exercise or not to exercise?
- exercise moderately but do not go to exhaustion
- aquatics are good. buoyancy
Spinal muscular atrophy (SMA)
- 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
Most common genetic cause of death in infancy?
SMA - spinal muscle atrophy
SMA - symptoms
-generalized muscle weakness, weak cry, trouble swallowing as well as sucking, and breathing distress. cannot sit without support
SMA - progression
-very rapidly
SMA type 1
(infantile-onset, werdnig-hoffmann disease)
- most severe form
- cannot sit independently
- between birth and 6 month old
- 50% die before 2nd birthday
SMA type 2
(intermediate SMA)
- affect babies before 18 months
- may be able to sit unaided or stand with support
- shortened life span
SMA type 3
(juvenile SMA, Kugelberg-Welander disease)
- mildest form
- between 18 months and late adolescence
- can stand and walk independently for some time
SMA type 4
(adult SMA)
- adult form of disease
- symptoms begin after age 35
SMA primary impairments
-muscle weakness secondary to progressive loss of anterior horn cells in spinal cord
SMA secondary impairments
- Cranial nerve involvement
- contractures
- muscle fasciculations
- respiratory distress
- scoliosis
- fatigability
SMA - treatment
-focus on feeding, ROM, positioning, respiratory care and selected developmental activities
