Myelodysplasia Part 1

Question 1

Myelomeningocele (MM)

Answer 1

open spinal defect, dorsally protruding sac containing meninges, spinal fluid, and neural elements

Question 2

Myelodysplasia

Answer 2

refers to defective development of any part of the spinal cord; the term Spina Bifida is the commonly used term referring to various forms of myelodysplasia

Question 3

Physical therapy for myelodysplasia

Answer 3

prevent secondary impairments contributing to functional limitations and participation restrictions

Question 4

What is neurological damage, loss of muscle activity and sensation determined by?

Answer 4

vertebral level

Expected mobility and independence affected by pattern of innervation

Question 5

Who is incidence highest in?

Answer 5

highest in Hispanic, lowest in black populations

Question 6

Etiology of myelodysplasia?

Answer 6

2-3% recurrence in siblings in US.
Fetal alcohol syndrome, valproic acid during pregnancy.
Inadequate dietary levels of folic acid.
Folic acid additive to enriched grain products
recommended dietary supplement for all women of childbearing age

Question 7

What is maternal screening looking for?

Answer 7

alpha-fetoprotein at 18 weeks

Question 8

What are the cranial signs in utero?

Answer 8

Lemon sign

Banana sign

Question 9

Lemon sign

Answer 9

overlapping frontal bones caused by enlarged ventricles

Question 10

Banana sign:

Answer 10

abnormally shaped midbrain and elongated cerebellum associated with Arnold Chiari malformation

Question 11

Hydrocephalus

Answer 11

Abnormal accumulation of cerebral spinal fluid, CSF in cranial vault
80% of cases of MM

Question 12

What is the primary cause of hydrocephaly in MM?

Answer 12

Arnold Chiari (Chiari II)

Question 13

Arnold Chiari malformation

Answer 13

cerebellar hypoplasia with caudal displacement of brainstem into foramen magnum

Question 14

What are red flags of shunt formation?

Answer 14

headache, fever/malaise, changes in appetite, weight.
altered speech, decreased activity level or school performance.
visual acuity, strabismus or diploplia; decreased visual-perceptual coordination.
seizures; increased spasticity, static or declining grip strength.
personality changes, e.g., irritability, difficult arousal

Question 15

Progressive Neurologic Dysfunction

Red flags

Answer 15

Gait deterioration or
loss of sensation or strength in extremities or trunk.
pain - repair site or radiating along dermatome.
onset or worsening spasticity.
development or rapid progression of scoliosis.
development unexplained lower limb deformity.
change in bowel or bladder sphincter control

Question 16

What are causes of progressive neurologic dysfunction?

Answer 16

shunt obstruction, lipoma, subarachnoid cysts of the cord.

tethering of spinal cord – traction on neural structures

Question 17

What is goal of PT with symmetric alignment?

Answer 17

minimize joint stresses and deforming forces, optimize muscle length for function

Question 18

What postures should be avoided?

Answer 18

habitual ‘frog-leg’, W-sitting, and crouch standing should be avoided

Question 19

Innervated muscle below the level of the lesion:

Answer 19

flaccid; spasticity(25%)

Question 20

When does positioning begin?

Answer 20

at birth: taught to parents, daily LE ROM exercises
splinting
manual exercise

Question 21

Overall goal of PT in MM?

Answer 21

prevent joint contractures, correct existing deformities, prevent or minimize effects of sensory and motor deficiency, and optimize mobility within natural environments

Question 22

What should be avoided:

Answer 22

excessive force causing fractures.

excessive hip adduction risking dislocation

Question 23

Measure hip extension in:

Answer 23

prone

Question 24

Measure ankle ROM:

Answer 24

subtalar neutral

Question 25

What contractures do full term infants have

Answer 25

``` hip flexion (30 degrees) knee flexion (10-20 degrees) ankle dorsiflexion (40-50 degrees) ```

Question 26

Pattern movements:

Answer 26

hip flexion, knee flexion with contralateral hip extension | hip flexion and abduction with holding at end ROM to increase ROM

Question 27

What can ROM restrictions result in?

Answer 27

ADLs; bed mobility, transfers poor postural habits, gait deviations risk for skin breakdown

Question 28

What should MMT look for on initial assessment?

Answer 28

level of muscle innervation baseline muscle function imbalances of power around joint prediction of future deformity aids decision making about orthotic

Question 29

How is motor level assigned?

Answer 29

according to last intact nerve root or the lowest level of antigravity movement through available ROM

Question 30

What does MMT grade 3 or better indicate?

Answer 30

muscle innervation

Question 31

What does strengthening in upper limbs do?

Answer 31

prepare for transfers, w/c propulsion, use of assistive mobility devices (AMDs).

Question 32

What does strengthening in the lower limbs do?

Answer 32

function within functional ROM, e.g., sufficient quad/HS strength (L4) may allow progress from KAFOs to AFOs

Question 33

When is the child at risk for loss of strength and endurance?

Answer 33

during rapid growth periods | post surgery

Question 34

Motor assessments:

Answer 34

Alberta Infant Motor Scale (AIMS). Peabody Developmental Motor Scales (PDMS-2). Gross Motor Function Measure (GMFM).

Question 35

What positions are static and dynamic balance observed?

Answer 35

sitting, four-point, kneeling, half-kneeling, standing, transitions

Question 36

Sitting in alignment:

Answer 36

hips and knees at 90o, feet resting on floor symmetrical weight-bearing, neutral pelvic tilt with slight lumbar lordosis, inclining seat backward 15 degrees minimizes lumbar stress, keeps pelvis properly seated

Question 37

Typical postural problems

Answer 37

Forward head, rounded shoulders, kyphosis. Excessive lordosis, anterior pelvic tilt. Rotation deformities of hip or tibia. Windswept hips, flexed hips/knees, pronated feet

Question 38

Pressure relief in children using wheelchair

Answer 38

wheelchair push-ups every 20-30 min. Seat cushion design. 50% of day in w/c in 4-5 year-olds

Question 39

Pressure relief in ambulatory children

Answer 39

Inspect lower limbs, skin under orthoses. | Regular orthotic checks/adjustments during growth spurts

Question 40

What can impairments in proprioception and kinesthesia affect?

Answer 40

balance and standing, more reliance on visual and vestibular inputs rather than information from feet and ankles for postural adjustments

Question 41

What do children rely on with a loss of distal balance control

Answer 41

reliance on support from stable objects, adaptive equipment

Question 42

Secondary Impairments of MM

Answer 42

MSK deformities joint contractures muscle imbalance upper limb deformities

Question 43

Spinal deformities are more common in:

Answer 43

higher level lesions

Question 44

What can severe kyphosis and scoliosis limit?

Answer 44

exercise tolerance, chest wall expansion restrict lung ventilation frequent infections

Question 45

Congential scoliosis:

Answer 45

vertebral anomalies | inflexible curve

Question 46

Acquired scoliosis:

Answer 46

muscle imbalance, flexible curve until skeletal maturity with little further progression

Question 47

Kyphosis

Answer 47

10-15% congential | 1/3 acquired by adolescence

Question 48

Thoracic to L2 Deviations:

Answer 48

hip flexion, abduction, ER contractures; knee flexion and ankle plantar flexion contractures; lumbar lordosis

Question 49

Low lumbar (L3-L5) Deviations:

Answer 49

hip and knee flexion contractures, increased lumbar lordosis, genu and calcaneal valgus, pronated foot position (WB); pronounced crouch gait, WB on calcaneus

Question 50

Sacral level deviations:

Answer 50

mild hip/knee flexion contractures, increased lumbar lordosis; varus or valgus ankle/foot with pronated or supinated forefoot; mild crouch gait, calcaneal WB’ing with plantar flexor weakness

Question 51

Crouch standing:

Answer 51

persistent hip/knee flexion | increased lumbar lordosis

Question 52

What is weak in crouch standing?

Answer 52

soleus weakness insufficient to maintain vertical tibia | orthopedic deformities-calcaneal valgus

Question 53

Secondary impairments to crouch standing:

Answer 53

secondary hip/knee flexion contractures in response to adaptive muscle shortening decreased torque-generating capacity

Question 54

Surgery candidate for hip subluxation/dislocation:

Answer 54

L3 or below with quad function. unilateral dislocation may interfere with sitting or standing posture, contribute to scoliosis, affect skin care. Painful hip dysplagia in ambulators

Question 55

What foot position is a priority?

Answer 55

plantigrade foot position in subtalar neutral

Question 56

What ankle and foot deformities are most common?

Answer 56

plantar flexor contracture | torsional deformities

Question 57

What level are ankle and foot deformities common?

Answer 57

L5 and above

Question 58

What deformity is seen in thoracic and high lumbar lesions?

Answer 58

ankle equinovarus | clubfoot

Question 59

What deformity is seen in sacral lesions?

Answer 59

Forefoot varus/valgus, supination or pronation, calcaneal varus/ valgus, pes cavus/ pes planus; claw-toe deformities

Question 60

What torsional deformities are seen:

Answer 60

excessive foot progression angle, windswept hips from femoral or tibial torsion