Myelodysplasia and Acquired SCI

Question 1

What is the most common source of SCI for all ages?

Answer 1

trauma

Question 2

t/f, children are much more likely to have an upper cervical spine injury than adults w/ no radiological evidence of bone damage

Answer 2

true, their spine is more mobile due to ligamentous laxity, shallow angulation of facets, incomplete ossification, and incomplete development of neck mms

Question 3

What level must a child be at least in order to be considered to do wheelies in a W/C?

Answer 3

7-8

Question 4

What are the different types of myelodysplasia (congenital SCI)?

Answer 4

Myelomeningocele, meningocele, lipomyelomeningocele, diastematomyelia, myelocystocele, sacral agenesis

Question 5

Which myelodysplasias are considered open, closed?

Answer 5

open- myelomeningocele, meningocele
closed- lipomas (lipomyelomeningocele)

Question 6

T/F, myelomeningocele usually do not have motor and sensory loss

Answer 6

false, DO tend to have motor paralysis and sensory loss

Question 7

T/F, meningocele are protrusions that are not skin covered and only contain CSF and nonfxnal and are associated paralysis

Answer 7

false, meningocele ARE skin covered (myelomeningocele not), and are NOT associated w/ paralysis

Question 8

t/f, lipomyelomeningoceles can either be with or without paralysis

Answer 8

true, can be either

Question 9

Which type of myelodysplasia tends to have a fibrous, cartilaginous or bondy spincule separating the SC into hemicords

Answer 9

diastematomyelia

Question 10

What are two things that can develop with myelodysplasia?

Answer 10

tethered cord (dura or skin attaches to the bone), dysplasia (abnormal tissue growth may cause pressure on nerves), these individuals are at risk for further loss of function over time

Question 11

Myelodysplasia is a disorder of ____ and ____.

Answer 11

mesoderm, ectoderm

Question 12

What term describes the development of SC below S2 vertebrae?

Answer 12

canalization (skin covered meningoceles, lipomas of the SC, myelocystocele)

Question 13

Which racial group is more likely to develop myelodysplasia/spina bifida? What are common teratogens? Nutritional deficiency?

Answer 13

celtics

FAS, street drugs, anti-seizure meds (valporic acid), Mexican women living along the Rio Grande River

Folic acid

Question 14

How can you diagnose spina bifida prenatally?

Answer 14

alpha-fetaprotein screening (WON’T detect skin covered lesions)
US- better at detecting open lesions
Amniotic fluid analysis

Question 15

T/F, can use ASIA on spina bifida/ MM (myelomeningocele) to determine level of motor paralysis

Answer 15

false, use international myelodysplasia study group criteria (IMSG) in this population but use MMT to be more specific

Question 16

t/f upper limb weakness occurs regardless of level of lesion in spina bifida

Answer 16

true, often a sign of progressive neurologic dysfunction

Question 17

What are the three ways motor paralysis can present in MM and which one is most common?

Answer 17

complete lesion, incomplete lesion, skip lesions -> common

Question 18

t/f, UE dyscoordination is very common in children with hydrocephalus

Answer 18

true, very common

Question 19

What are three possible causes that contribute to UE dyscoordination in children with hydrocephalus?

Answer 19

cerebellar ataxia related to Arnold chiari II
Motor cortex and internal capsule damage due to hydrocephalus
motor learning deficits due to use of arms for support and balance rather than manipulation

Question 20

t/f, children w/ spina bifida tend to have normal scores in tests that require hand-eye coordination

Answer 20

false, tend to have lower scores that might be contributed to visuoperceptual deficits

Question 21

t/f, children with spina bifida may have delayed development of hand dominance

Answer 21

true, many are mixed dominant or LUE dominant (possibly due to l hemisphere damage)

Question 22

What is your main concern when looking at tone when dealing with a pt with spina bifida?

Answer 22

pt can range from flaccid to spastic with mostly LMN with scattered UMN, be aware of any emergence of UMN signs in a child where there was none previously or an increase in spasticity or other UMN signs as this requires URGENT attention!

Question 23

Fill in the blank: fewer than ___% of children with MM develop voluntary control of urinary or anal sphincter
Bowel training before age __ -better compliance and improved outcomes

Answer 23

5, 7

Question 24

What is your main goal when dealing with a neurogenic bladder in a child with MM?

Answer 24

infection free social continence and preserve renal function
inadequate emptying + urinary retention + reflux = renal failure and infections

Question 25

What are the “biggies” in terms of impairments for kids with spina bifida to be aware of?

Answer 25

seizures, hydrocephalus, arnold chiari malformation, tethered cord, progressive neurological dysfunction

Question 26

You have a pt with spina bifida who is slurring their words, seem irritable and more quiet than normal. Their parent says they have been performing worst in school, recently wet the bed after years of not, and notice that they are not eating like normal, what are you concerned with at this point?

Answer 26

child may be having a shunt dysfunction

Question 27

You have a spina bifida pt who is having increased spasticity, increased pain, and change in bowel and bladder function, what do you think might be happening?

Answer 27

tethered cord

Question 28

You have a kid with spina bifida that has been complaining of neck pain, difficulty swallowing, balance issues, decreased UE coordination and headache that worsens when they cough or strain, what do you want them to be checked for?

Answer 28

arnold chiari malformation II (caudal displacement of hindbrain through foramen magnum)

Question 29

What are some symptoms that would indicate progressive neurological dysfunction?

Answer 29

altered gait, onset or progression of spasticity, decline in motor or sensory fxn, change in b+b fxn, rapid progression of scoliosis, fever, malaise and irritability etc

Question 30

t/f, incidence of fractures is less in children with spina bifida than in the non-paralyzed population

Answer 30

false, it is much greater due to osteoporosis and impaired bone growth and development

Question 31

What are reasons that explain why children with spina bifida are more susceptible to fractures?

Answer 31

decreased bone mineral density
decreased length and width of bones
1. hypotonic or flaccid mms result in DECREASED MM PULL on bones
2. DECREASED LOADING of long bones from altered mobility

Question 32

Fill in the blank: normal infants have ____ shaped tibias while infants with LE paralysis have ___ shaped tibias that do not change shape as the infant grows

Answer 32

triangular, circular

Question 33

describe the mechanism of acquired scoliosis

Answer 33

concave shortening of soft tissues-> cartilage degenerates on concave side-> disc is displaced toward convexity-> bony deformity as there is decreased growth on concave side due to compression and overgrowth on convex side due to minimal loading = vertebral wedging -> rotary alterations leading to facette incongruency

Question 34

describe the mechanism of development of kyphosis in a child with spina bifida

Answer 34

lengthening of posterior structures and shortening of anterior structures as paraspinal mms are displaced laterally with decreased innervation and may end up anterior to the vertebrae w/ progressive kyphosis

Question 35

in managing subluxations in spina bifida, reduction is NOT a prerequisite for ambulation and is done in walkers if: (2 things)

Answer 35

children < L3 w/ good quad fxn
good potential for remaining fxnal ambulators

Question 36

What is the significance of hip contractures in pts with spina bifida?

Answer 36

increase the cost of movement
high L and T (lesion) -> unopposed iliopsoas
in ambulating kids- compensation for knee flex, lordosis and head forward posture (crouch to compensate for posterior weakness)

Question 37

What knee deformities are common in spina bifida?

Answer 37

knee flexion contractures (esp in w/c users and high level injuries and low lumbar with crouched gait)
valgus (enhanced W sitting for stability and common in lesions below T12)

Question 38

t/f, the majority of kids with spina bifida tend to have some foot deformity

Answer 38

true, club feet at t and high L lesions, claw toes in sacral lesions, occur in both ambulatory and non ambulatory children

Question 39

achieving ____ foot is critical for children with spina bifida

Answer 39

plantergrade

Question 40

What are indications for treating gait in MM w/ active PT intervention?

Answer 40

child first learning to walk, progressing to a new assistive device or orthotic, progressing to a more efficient gait pattern, potential to improve gait, improve safety, more advanced walking activities

Question 41

What is the main predictor of ambulation potential in child with spina bifida?

Answer 41

quadriceps strength followed by iliopsoas strength (4-5 w/ some quadriceps to be community ambulators)