Exam 2

define function

quantitative aspect

achievement of skill

define performance

qualitative aspect

how well skill was executed

define evaluative measure

responsive to change in motor behavior over time

define discriminative measure

seeks to differentiate pediatric clients with typical vs atypical development

what do we have to observe in quality of movement in WB surface

BOS area (wide, narrow, adequate)

COM (high, low, anterior, posterior)

use of support surface (posture and movement)

define alignment

adjustment or arrangement of parts or segments of the body in relation to each other

what 2 things do we look for when analyzing antigravity movement

strategy (dominance/absence, mechanical/active)

quality (elongation on WB side)

what does coordination include

timing

velocity

direction

force

amplitude

define dissociation

isolated movement of one part of the body independent of another part

define muscle tone

muscle’s resistance to passive stretch

how does symmetry develop

because asymmetry is variable in the first 2-3 months of life

how is visual exploration affected in CMT

asymmetrical

define torticollis

persistent and predictable asymmetrical posturing of the head and neck

define CMT

postural condition evident shortly after birth characterized by lat flex to one side and cervical rotation to the opposite side and is usually associated with unilateral SCM restriction

what are some of the hypothesized causes of CMT

intrauterine malpositioning

birth trauma

what are some of the nonmuscular etioligies of CMT

bony

inflammatory

soft tissue

neurological

ocular

plagiocephaly

twisted skull

parallelogram shape

torticollis is the most common associated finding with what

deformational plagiocephaly

what caused higher incidence of plagiocephaly in the 90s/2000s

safe to sleep program

recommendation to put infants to sleep on their backs

what is the target tummy time by 7 weeks

at least 15-30 min total daily

what offsets the negative effects of back sleeping for motor skill acquisition

> 1 hour of cumulative tummy time per day

what are the 3 types of CMT

SCM mass

muscular

postural

define SCM mass CMT

palpable mass

intramuscular fibrotic thickening of the SCM

passive and/or active ROM limits

define muscular CMT

SCM tightness without palpable mass

passive ROM limits

define postural CMT

postural preference

no passive ROM restrictions

what is used to grade CMT

CMT-SCS (severity classification system)

what is NOT part of the CMT-SCS

degree of lateral tilt

what are typically seen in Argenta V

temporal bulging

abnormal vertical growth (cone shaped)

what is common with Argenta IV

ipsilateral facial asymmetry

what is common with Argenta I

one sided occipital flattening with opposite side forehead flattening

what is common with Argenta II

one sided ant ear shift

what is common with Argenta III

one sided forehead frontal bossing

what are the 5 first choice interventions for CMT

1. neck PROM

2. neck and trunk AROM

3. development of symmetrical movment

4. repositioning/environmental adaptations

5. parent/caregiver education

prognosis of CMT is most influenced by what

age at onset of PT

DMD is linked to what

X recessive

affects males

what is different about SMA and DMD genitically

males and females are affected equally in SMA

DMD is (progressive or stagnant)

progressive

what is the mechanism of the disease in the gene for DMD

mostly deletions in the dystrophin gene

how is DMD usually discovered

creatine kinase increased on blood test (most at 5 yrs old)

what is dystrophin at the muscular level

stabilizes structural membrane

stress protector

where is dystrophin found other than muscle

heart

brain

smooth muscle

what happens if dystrophin is not present

muscles susceptible to injury

what is stage 1 of DMD

pre-symptomatic

no gait disturbance yet

CK can be found elevated by chance

what is stage 2 of DMD

early ambulatory

waddling gait

Gower’s maneuver

may be toe-walking

what is stage 3 of DMD

late ambulatory

increasingly labored gait

losing ability to climb stairs and rise from floor

what is stage 4 of DMD

early non ambulatory

may develop scoliosis

may be able to self propel for some time

able to maintain posture

what is stage 5 DMD

late non ambulatory

UE function and postural maintenance are increasingly limited

what is characteristic of early ambulatory (stage 2) DMD

calf pseudohypertrophy

muscle is replaced with fatty tissue

DMD progresses from…

proximal to distal

70% of pts in late ambulatory (stage 3) develop what

contractures in: heel cord, ITB, and hip joints

what is weaker (muscle imbalances) in late ambulatory (stage 3) DMD

ankle PF and invertors > ankle DF and evertors

neck ext > flexors

deltoids > biceps and triceps

wrist flexors > ext

knee flexors > ext

at what stage does a DMD patient need a WC

early non ambulatory (stage 4)

what age are pts typically at stage 4 early non ambulatory phase

starting at 8-9 yrs old

what incidence increases with age in DMD patients

cardiomyopathy disease

what is the median survival age in DMD patients

20s

what stage do we need to focus on ROM in ankle and hip

early non ambulatory phase

what are standard of care in the early ambulatory phase (orthotics)

dynamic AFOS at night

what is standard of care in the non ambulatory phase (orthotics)

solid ankle AFOs for positioning during the day

what is used in later phases of DMD (orthotics)

wrist and hand splints for long and wrist finger flexors/extensors

what is implemented in the late ambulatory phase (orthotics)

standers (standing frame)

when does scoliosis typically progress in DMD patients

non ambulatory phase

what are some DMD specific OM

NSAA (north star ambulatory assessment

PUL (performance of upper limb)

in the early ambulatory phase, what interventions are we focusing on

swimming

stretching (ankle PF + hip flexors focus w caregiver education)

resting AFOs to maintain mobility (but not for ambulation)

use scooter/stroller/WC for long distances to conserve energy

in the late ambulatory phase, what interventions are we focusing on

focus on ROM and independence

stretching (ankle PF + hip flex + knee flex + lumbar ext)

KAFOS for standing and flexibility

standing devices

ultra lightweight WC

recommend power WC

in the early nonambulatory phase, what interventions are we focusing on

attention to flexibility of UE (wrist and finger flex + elbow flex + shoulders)

resting hand splints

monitor for scoliosis

in the late non ambulatory phase, what interventions are we focusing on

equipment to support independence and participation (ex: power adjustable bed)

what is the general recc for exercise in DMD patients

submax

aerobic and strengthening

swimming and cycling

what do we avoid when treating DMD pts

high resistance exercise and high intensity eccentric

what is the medical standard of care in DMD patients

corticosteroids

what happens with corticosteroid use in DMD patients

improved strength and function (12 months)

improved strength (up to 2 years)

what are some of the side effects of cortiocosteroids in DMD treatment

weight gain

Cushingoid features (moon face)

stunted growth

delayed puberty

behavioral changes

immune suppression

bone demineralization

define SMA

genetically determined motor neuron disease

what is the common genetic etiology of SMA

homozygous deletion of SM1 on chromosome 5

what is the most severe and common type of SMA

type I

identify type I SMA

severe

limited life expectancy

respiratory failure

identify SMA type II

intermediate

sitting or standing

life expectancy shortened

skeletal deformities

identify SMA type III

mild

walk at some point

nearly normal life expectancy

proximal weakness

what is special about SMA

SMN-2 gene (can have many copies) = backup to produce SMN protein

uniquely human

what explains the range os SMA types

SMN2 copy number determines SMA severity (4 for type III, 2-3 for type II)

how is SMA treatment for PT organized

symptoms of SMA type are ranked and addressed in order of importance

what are the 3 most important (at the top of the scale) to address in treatment of SMA patients

muscle weakness

postural control

contractures (limb, neck, jaw)

what is the most important evaluation for non sitters SMA (type I)

CHOP INTEND

what is the most important evaluation for sitters SMA (type II)

hammersmith expanded

revised UE scale

handheld dynamometry/MMT

forced VC

what is the most important evaluation for walkers SMA (type III)

6MWT

hammersmith expanded

hand held dynamometry/MMT

forced VC

what is included in the CHOP INTEND

active and elicited reflexive movement

assessments of head, neck, trunk, and limb strength

who is the CHOP INTEND made for

weak infants

what is NOT included in CHOP INTEND

respiratory or feeding

hammersmith functional motor scale was initially created for which SMA type

type II (sitters)

*has ceiling effect for walkers (type III)

what is typically found in the 6MWT in SMA pts

17% change in velocity from minute 1 vs minute 6

what is the most important intervention in type I SMA (nonsitters)

stretching

ROM

what is the most important intervention in type II SMA (sitters)

stretching

ROM

what is the most important intervention in type III SMA (walkers)

exercise

what is reccomended at 12 months of age in SMA type II (sitters) and why

stander

this is the age that they would most likely be starting to stand

what is not common for SMA type III when walking

thoracic bracing

what are the strengthening guidelines for SMA pts

submax

all types (eccentric, concentric, etc)

BUT no resisted eccentric exercises for weaker muscles or for neck musculature

what is the hypothesized pathophysiology of DCD

altered brain development

disrupted connection w sensorimotor regions and areas allowing mental imagery, memory recollection, and multimodal sensory integration

DCD suspected when you see what 3 things

1. motor impairments and delay in skills (not age-appropriate)

2. sufficient opportunity to learn the skill

3. nothing else causing impaired coordination

what are the 4 things that u need to meet DCD criteria

1. motor performance deficits

2. participation and ADL deficits

3. early onset

4. no exclusionary conditions

what things are commonly associated with DCD

ADHD

autism

speech impairments

learning or intellectual disability

if someone has a freq associated condition, what is the likelihood they also have DCD

50%