Myelodysplasia

Question 1

Myleodysplasia

Answer 1

Defective development of any part of the spinal cord

Question 2

Spina bifida

Answer 2

Most commonly used to describe various forms of defects of neural tube closure

Question 3

Aperta

Answer 3

Visual or open lesion

Question 4

Occulta

Answer 4

Hidden or not visible lesion

Question 5

Myelomeningocele

Answer 5

External protrusion of meninges and spinal cord

Not covered by skin

Question 6

Meningeocele

Answer 6

External protrusion of meninges

Question 7

Diastematomyelia

Answer 7

Fibrous, cartilaginous or bony band separating the spinal cord into hemicords each surrounded by dural sac

Question 8

Lipoma

Answer 8

Occulta, but usually visible subcutaneous fat mass
Classified by location
Lipomyelomeningocele w/ paralysis
Lipomeningocele w/ no paralysis
Lipoma of the filum terminal
Lipoma of the Claudia equina or conus medullaris

Question 9

Upper end of neural tube usually closed on

Answer 9

25th day

Question 10

Bottom of the neural tube usually closed

Answer 10

27/28 day

Question 11

Spina bifida is the result of

Answer 11

The neural tube failing to develop or close properly

Question 12

Myelomeningocele: genetics

Answer 12

Recurrence 2-3% risk in siblings
Associated w/ “syndromes”
High variability in incidence among various ethnic groups

Question 13

Myelomeningocele: teratogens

Answer 13

-maternal alcohol consumption
Valproic acid
Street drug abusers

Question 14

Myelomeningocele: nutritional deficiencies

Answer 14

-inadequate folic acid!! 
Begin supp at least 3m before conception
-reduce risk 70%
-w/ fam history/1st deg relative - 4mg/day
W/out fhx 0.4mg/day

Question 15

Internationally: % of pregnancies with prenatal dx of neural tube deficits are terminated

Answer 15

23%

Dx usually occurs at 18 weeks

Question 16

Myelomeningocele DX:

Answer 16

Maternal alpha-fetoprotein screenings, US, amniotic fluid analysis
-lemon sign, banana sign

Question 17

Myelomeningocele: perinatal management

Answer 17

Prenatal dx allows for repairs in utero

Post natal closure of tube requires sx w/in 48 hours to avoid infection and drying of nerve roots

Question 18

Myelomeningocele musculoskeletal deformities

Answer 18

Spinal and LE limb deformations and contractures

Restriction in UE ROM due to overuse for WB and poor posture

Question 19

Myelomeningocele typical postural deficits

Answer 19

Forward head, rounded shoulders, kyphosis, scoliosis, excessive lordosis, ant pelvic tilt, rotational deformities of hip or tibia, flexed hips and knees, pronated feet

Question 20

Myelomeningocele: thoracic to L2

Answer 20

Hip flex, AB, ER contracture
Knee flex contract
PF contract
Lordotic lumbar spine

Question 21

Myelomeningocele: L3-L5

Answer 21

Hip and knee flex contracture
Increased lumbar lordosis 
Genu and calc valgus
Pronated feet
Often walk in crouched gait and bear weight through calcaneous

Question 22

Myelomeningocele sacral level

Answer 22

Mild hip and knee flexion contracture
Increased lumbar lordosis
Foot in varus or valgus w/ pronated and supinated foot
Mild crouching gait

Question 23

Myelomeningocele osteoporosis

Answer 23

Decrease bone mineral density

• walking ability highly correlated to increased bone mineral density
• fax may not be immediately apparent due to poor sensation

Question 24

Myelomeningocele motor paralysis

Answer 24

Motor level - lowest intact functional NM seg

May be asymmetric

Question 25

Myelomeningocele sensory deficit

Answer 25

Don’t always correlated to motor levels Eval Pt ed

Question 26

Myelomeningocele hydrocephalus

Answer 26

At birth 25% or more Additional 60% develop after sx closure of lesion 80-90% require CSF shunt

Question 27

Myelomeningocele cog function

Answer 27

W/ or w/out uncomplicated hydrocephalus, intellegence typically average

Question 28

Myelomeningocele latex allergy

Answer 28

73%

Question 29

Myelomeningocele seizures

Answer 29

10-30%

Question 30

Myelomeningocele skin breakdown

Answer 30

Decubitus ulcer and other breakdown occur in 85-95%

Question 31

Myelomeningocele cranial nerve palsy

Answer 31

May result from chiari malformation, hydrophealus, dysphasia of brain system

Question 32

Myelomeningocele neurogenic bowel

Answer 32

<5% have control of urinary or anal sphincter

Question 33

Myelomeningocele neurogenic bladder

Answer 33

Early catheterization will help avoid overstretching bladder wall Can learn to self cath by 6-8yo

Question 34

Myelomeningocele exam

Answer 34

``` ROM WNL Strength, GM, FM, ADL Sensation Home and school enviro *delays in GM, use of AD to facilitate exploration, social, ADLS *monitor joint alignment, strength, contracture, posture *tethered cord ```

Question 35

Myelomeningocele common goals across life span

Answer 35

Prevent joint contracture Correct existing deformities Prevent/min effect of sensory and motor deficiency Optimize mobility w/in natural enviro

Question 36

Myelomeningocele outcome T10 above

Answer 36

Strong UE, upper TSpine, neck motions | Weak lower trunk, diff w/ sitting and resp

Question 37

Myelomeningocele outcomes T12

Answer 37

Strong trunk and sitting balance | May have weak hip hike form QL

Question 38

Myelomeningocele outcomes: general thoracic

Answer 38

W/c Tend to have greater CNS involve- cog impairment Supervision required in living situation May work in sheltered settings or volunteer work

Question 39

Myelomeningocele L1-L2

Answer 39

L1 weak hip flex L2 hip flex, add, rotators 3/5 or higher -frequent dislocation of hip. Due to unopposed flex or ad Household amb for small children poss w/ support/orthotics W/c for community 50% live (I)

Question 40

Myelomeningocele L3

Answer 40

``` Strong hip flex, weak rotation Some knee ext KAFO and crutches for household in childhood W/c for community 60% (I) ```

Question 41

Myelomeningocele L4

Answer 41

Calc deformities common - unopposed ant tib Strong knee ext Funct amb w/ AFO and crutches (20% amb as adult) Focus on maintaining ankle joint alignment

Question 42

Myelomeningocele L5

Answer 42

``` Lateral hamstring 3/5 and either -glut min 2, post tib 3, peroneus tertius 4 Antigravity knee flex and weak hip ext Require orthoses for alignment Bilateral UE support recommended 80% achieve (I) living ```

Question 43

Myelomeningocele S1

Answer 43

Gast/sol 2 Glut med 3 Glut max 2 Amb w/out orthotics for support

Question 44

Myelomeningocele S2, S2-3, and “no loss”

Answer 44

S2- decreased push off and stride length w/ running S2-3 more LE have 5, few 4 “No loss” normal BB function, normal strength

Question 45

Myelomeningocele intervention

Answer 45

Children encouraged w/ high dose or normal developmental activities in at risk areas Remediation - rep of graded task in area of concern Reach compensatory skills - inc independence

Question 46

Myelomeningocele strengthening indicated if

Answer 46

Function is present Weakness in muscle group important for postural stability, ADL, mobility or balance of muscle forces around joint Strengthen w/in functional ROM

Question 47

Myelomeningocele mobility: measure energy expenditure via

Answer 47

HR

Question 48

Myelomeningocele indications for gait training

Answer 48

Potential for progression to new orthosis or AD Potential for improving pattern Improve safety and confidence Efficiency of gait and transfer

Question 49

Myelomeningocele: key muscle groups for amb

Answer 49

``` Iliopsoas Glute med and max Quad Ant tib Hamstring ```