11
- 51% to 65% or 80-90% of infants
- About 25% have evidence at birth
- 55% will develop symptoms within first several days
- 10% will need a shunt placed within first 6 months
Hydrocephalus
- Common because innervations at sacral level
- Kidney problem - difficulty emptying bladder
- Hydronephrosis - result of a spastic bladder wall and/or a spastic urethral sphincter
- Management of bowel and bladder secretions critical
- Prevent social problems associated with incontinence
- Transfer and fine motor skills needed to manage bowel and bladder
- Clean intermittent catheterization
- Socially acceptable continence achieved by 74% of individuals with MM
Impairments in bowel and bladder control
- Headache
- Irritability
- Fever unrelated to illness
- Nausea
- Increased spasticity in innervated muscles
- Problems with vision
- Problems with speech
- Increased difficulty with postural control
- Decreased performance in school
- Decreased level of consciousness
Signs of shunt malformation *** (CAN LEAD TO DEATH)
- Change in sensation or continence
- Inability to perform or difficulty performing tasks that the child was previously capable of performing
- Spasticity in muscles with sacral nerve roots
- Development of scoliosis at a young age
- Reduced activity level tolerance
- Changes in muscle tone and further paralysis of previously innervated muscles
Signs of tethering cord
Which symptom is an emergent referral to the hospital if the patient has a VP shunt?
Nausea and vomiting ***
- As a child grows and develops
- Development of adhesions or bony spurs at lesion closure site
- Do not allow the SC to slide normally
- Excessive stretch to spinal cord can cause metabolic changes and ischemia of neural tissue with degeneration of muscle function
Tethered spinal cord
- May occur in children with MM
- Higher in children with hydrocephalus
- Majority of children without hydrocephalus or with uncomplicated hydrocephalus will have normal intelligence
- Higher intelligence score in lumbar and sacral lesion groups versus thoracic
- Normal distribution curve of average IQ but shifted to the left about 20-point lower IQ
- Higher the level of injury = lower the cognitive status
Intellectual and visual perceptual deficits
- Excessive accumulation of fluid dilating cerebral ventricles after primary spina site is closed
- Controlled via placement of ventricular-peritoneal (VP) shunt
- Plastic catheter excess CSF from ventricles into peritoneal cavity
- Shunt malfunction: shunt clogged, shift or dysfunctional
- common cause of death if unrecognized
Hydrocephalus
- Social cognition
- Phonological processing
- Long term memory
- Expressive language
- Reading
Area of strength in cognition for MM
- Speed of processing
- Immediate registration
- Learning and memory
- Organization
- high level language
- Complex and less structured tasks
- Cognitive and visual perceptual deficits
- Delayed recall of recently acquired word
- Slowed acquisition of new words
- Attention/organizational deficits
- Abstract reasoning
- Visual-perceptual abilities
- Eye-hand coordination
- Visual motor integration
- Writing
- Mathematics
Cognitive and visual perceptual impairments in MM
- 10-30% of children with MM
- Associated with brain malformation
- CSF shunt malfunction or infection
- Residual brain damage from shunt infection
Seizures
Lesions below ___ are more likely to have IQs over 80, walk, be independent, drive, and be employed
L3 ***
- More prevalent in children with myelodysplasia than other pediatric neurological conditions
- 0-22% or 18-40%
- Significant concern
- balls, WC seats and tires, braces, balloons, gloves, toys
- Anaphylaxis: life threatening
- PT should educate families to avoid exposure
Latex allergies
- Thoracic and high lumbar level lesions might start walking: < 20% still walking by age 9
- Mid lumbar level lesions: 40-70% are still walking at 9 years
- Sacral lesions: typically reach and maintain functional ambulation with AD and/or orthotics
Potential for ambulation in MM
- 85-95% by the time the individual reaches adulthood
- Tissue ischemia from excessive pressure
- Thoracic > high lumbar > lower lumbar/sacral
- Lower limb breakdown occurs evenly across lesion levels
- Friction burns from crawling and scooting
- hot water scalds
- Pressure from ulcers from orthotics
Skin breakdown
Tell me motor function level:
- LE movement: No voluntary movements of the legs
- Possible orthotic: parapodium for exercise if thoracic 12 motor function
- Indications: upright positioning
- Ambulation: walking for exercise only; WC for mobility
Thoracic
Tell me motor function level:
- LE movement: all the above + antigravity knee flexion, ankle DF (grade 4), Ankle inversion might be present
- Possible orthotic: AFO and forearm crutches (may need KAFO and walker when first learning to walk); patellar tendon bearing, ground reaction force orthotic design
- Indications: medial and lateral knee or ankle instability; insufficient knee extension moment; inadequate toe clearance; crouched gait
- Ambulation: short distances
Lumbar 4
Tell me motor function level:
- LE movement: strong hip flexion and adduction; weak hip rotation; antigravity knee extension
- Possible orthotic: KAFO with forearm crutches
- Indications: medial and lateral knee instability; weak quads (grade 4 or less)
- Ambulation: household and short community distances (as children)
Lumbar 3
Tell me motor function level:
- LE movement: all the above + hip abduction, weak hip extension, weak hip abduction, antigravity knee flexion, strong ankle DF with eversion, weak ankle PF with inversion, weak toe extension
- Possible orthotics: orthosis required to correct foot alignment (SMA orthosis or shoe orthotic), forearm crutches recommended
- Indications: lack of or ineffective push off, gluteal lurch, increased energy expenditure, prevention of foot deformities or skin breakdown caused by unequal weight distribution, poor alignment of subtalar joint, forefoot, or rear foot
- Ambulation: community ambulation
Lumbar 5
Tell me motor function level:
- LE movement: weak hip movements; L1: maybe weak hip flexion; L2: hip flexion, adduction at grace 3 or better
- Possible orthotic: RGO or KAFO using upper limb support
- Indications: unable to maintain an upright posture with hips extended; RGO indicated to facilitate hip extension and swing phase
- Ambulation: short distance household ambulation as children; WC in the community
Lumbar 1-2
Tell me motor function level:
- LE movement: all the above + hip extension (grade 2 or 3), knee flexion, weak ankle PF, flexion and eversion, toe flexion
- Possible orthotics: non required, SMA or shoe orthotic recommended to improve alignment, use of crutches to improve pelvis and hip kinematics
- Indications: medial and lateral ankle instability
- Ambulation: community
Sacral 1
Tell me motor function level:
- LE movement: most lower limb muscle groups at a grade 5 with only a few groups at grade 4
- Possible orthotics: foot orthoses
- Indications: to maintain ankle in subtalar neutral position; to optimize ankle muscle length
- Ambulation: community
Sacral 2-3
Tell me motor function level:
- LE movement: all the above + ankle PF grade 3, hip extension grade 4
- Indications: decreased push off and stride length during fast walking or running
- Ambulation: community
Sacral 2