Pedi

Question 1

Outcome Measure: Denver

Answer 1

norm-referenced
screening tool
0-60 months

Question 2

Outcome Measure: PDMS 2

Answer 2

Peabody
norm & criterion referenced
0-72 months
provides standard scores, % ranks, & age equivalents

Question 3

Outcome Measure: BOT-2

Answer 3

norm-referenced
4-21 years
53 items total: running speed, agility, coordination, etc..
provides age-based standard scores, % ranks, & age equivalents

Question 4

Outcome Measure: GMFM

Answer 4

criterion-referenced
*designed for children with CP
5 months - 16 years
provides % rank
5 domains: lying/rolling, crawling/kneeling, sitting, standing, walk/run/jump

Question 5

Outcome Measure: Weefim

Answer 5

pedi version of FIM
criterion-referenced
6 mo - 8 years
18 items (0-7)

Question 6

Outcome Measure: Pediatric Balance Scale

Answer 6

modified Berg for pedi
criterion-referenced
5-15 years

Question 7

Norm-referenced

Answer 7

standard point score
evaluates individual performance against group
normal distribution of scores
summative

Question 8

Criterion-referenced

Answer 8

cut-off scores (range)
performance against standard
content specific
discriminative
provides info for tx plan
formative

Question 9

GMFCS (Gross Motor Function Classification System)

Answer 9

Categories of severity:
Level I: walks without restrictions, limitations are more advanced
Level II: walks without AD, limitations outdoor/community
Level III: walks with AD, limitations outdoor/community
Level IV: power mobility with community navigation
Level V: self mobility severely limited, even with use of AD/power mobility

Question 10

Spina Bifida Occulta

Answer 10

open vertebral arch, NO herniation (most common)

Question 11

Meningocele

Answer 11

unfused arch, meninges protrude, spinal cord intact

Question 12

Myelomeningocele

Answer 12

unfused arch, meninges AND spinal cord protrude

abnormal CNS function at/below level

Question 13

What kind of mobility is expected with myelomeningocele at L1-2? S1?

Answer 13

L1-2: household ambulation

S1: community ambulation

Question 14

Arnold Chiari malformation is present in what % of myelomeningocele?

Answer 14

80%

Question 15

APGAR stands for…

Answer 15

A=activity (muscle tone)
P=pulse
G=grimace (reflex)
A=appearance (color)
R=respiration

Question 16

Prognostic Factors for future independent walking in CP: (4)

Answer 16

1. Head control by 9 months
2. Rolling supine>prone by 18 months
3. Sitting without UE support by 24 months
4. Reciprocal crawling by 30 months

Question 17

Type I SMA also known as __

Answer 17

Werdnig-Hoffman
onset: birth - 6 months (have read 3-9)
death usually within 1st year

Question 18

Type III SMA also known as __

Answer 18

Kugelberg-Welander

Question 19

Becker Muscular Dystrophy

Answer 19

milder form, symptoms begin 8-10 years, most have normal life span

Question 20

S/s associated with DMD

Answer 20

• walking delayed >18 months
• frequent falls
• postural deviations: lumbar lordosis, scapular winging, scoliosis
• Gower’s sign
• gastroc hypertrophy
• toe walking
• wide-based, waddling gait

Question 21

Cognitive dysfunction present with __% of individuals with DMD

Answer 21

33%; 2* dystrophin isoforms expressed in the brain

Question 22

Duchenne Muscular Dystrophy (DMD) typically age of dx

Answer 22

2-5 years

Question 23

Leading cause of death with DMD

Answer 23

Respiratory complications

Question 24

How do you diagnose DMD?

Answer 24

genetic testing (mutation to Dystrophin gene on the X chromosome at Xp21)
mm biopsy
elevated CK levels

Question 25

Common Rx for DMD

Answer 25

Glucocorticosteroids (Prednisone)

• adverse effects: weight gain, vertebral fx (~1/3 of long-term users)
• recommended for ALL ambulant cases (4-6 years) & in most older ambulant children

Question 26

DMD Life Expectancy

Answer 26

20-25 years, up to 40s with NIV (non-invasive ventilation)