Myelodysplasia

Physical Therapy 5410 with Pardo at Wayne State University

About this deck

By: Ashley DesJardins
Created: 2012-02-13
Size: 58 flashcards
Views: 40

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Definition of Myelodysplasia

Defective development of the spinal cord related to two diff processes in the developing NS abnormal neutralization or canalization.

Early diagnosis includes:

Quad screen which includes Alpha Fetal Protein (16 weeks gestation)

Detailed Ultrasound

Amniocentesis (less common)

What involves the non fusion of the vertebral arches/meninges or neural tissue but underlying neural tissue is unaffected?

Spina bifida occulta

Thought to have no neurological dysfunction

Recently been associated with urinary tract disorders.

**only distinguished by pigmentation, dimple, or tuft of hair in location

This disorder has visual or open lesion to spinal cord, with many variations w/ diff incidents of neurosensory loss

Spina bifida open or cystica

Meningocele (just meninges, no punching of neural tissue)

Myelomenigocele (spinal tissue, disruption)

Incidence: Approx __ per 1000 births. __ most common birth defect. Increase in siblings of up to __%. What populations are most affected?

1/1000. 2nd most. 5%

HIgher in Irish and Celts

China 10/1000

Japanese 3/1000

Urban hispanic and African Americans are increasing (due to nutritional and environmental factors)

Is there a definitive cause for myelodysplasia?

What are some possible causes related to genetic, environmental and dietary factors.

Genetic abnormalities/chromosomal abnormalities & syndromes

Dietary - decrease folic acid at conception =Neural tube deficits; alcohol consumption.

Environmental - maternal hyperthermia (sauna)

Increase if mothers obese, diabetic, on antiseizure meds

Prognosis: __% survival perinatally due to __ __. Prevalence has __ due to increased survival.

90%, improved medical management (antibiotics, ventricular shunts and urinary management), increased.

Survival into adulthood is ~85%

How would you repair a lesion?

Surgical placement of neural tissue and meninges within the spinal canal.

Usually done within 24-48 hours of birth (prevents infections and limit the neurological injury)

Can a shunt and closure be performed at the same time?

Yes.

Fetal (prenatal) surgery has these potential outcomes:

decreases continued neurological damage occurring during the last months of pregnancy.

decrease hindbrain herniation

Decrease hydrocephalus

What hospitals are presently conducting RCT of fetal surgery?

Children's Hospital of Philadelphia

Vanderbuilt

University of California SF

Name some complicating conditions: (4)

Hydrocephalus

shunt dysfunction

Arnold Chiari II

Cranial nerve palsies (VI, IX, X)

Info about hydrocephalus...

25% born with this, add. 60% more will develop after surgical closure.

Associated with arnold chiari II

over 80% require CSF shunting (can be externally programmed to adjust the flow in shunt, variety of ventriculoperitoneal shunting procedures)

Fever, malaise, vomiting/change in appetite, recurring headache, changes in speech, decreased school performance/memory, are symptoms for what?

Hydrocephalus or shunt dysfunction

More: incontinence worsens, difficulties w/ visuomotor/perception, decreased activity level/difficult to arouse in morning, increase irritability, onset or increase incidence or seizures, edema&redness along shunt.

Malformation of cerebellum, pons resulting in caudal displacement of the brain stem through foramen magnum is...

Arnold Chiarii II

CSF flow is disrupted leading to hydrocephalus

possible compression of cranial nerves and brain stem (if severe enough)

Stretch pressure on brachial plexus (if severe enough)

*diff. reasonings for this disease (first two mentioned)

Management of Arnold Chiari depends on __ & __. Can lead to __. outcomes appear better with __ __. What does surgery involve?

severity & progression. Mortality. Early surgery.

Surgery involves decompressing brain stem (remove part of lamina in upper cervical area, may decompress the posterior fossa in older individuals)

Cn VI oculomotor weakness in __ __. CN IX and X = __ & __ dysfunction. What is the management for these CN palsies?

lateral recuts. Pharyngeal and laryngeal dysfunction.

Management - neurosurgical shunting/decompression may be necessary with mixed impact on CN palsies.

Progressive Neurological dysfunctions with myelodysplasia include...

Hydromelia

tethered spinal cord

scoliosis

Definition, symptoms, and management for hydromyelia include:

Excessive CSF accumuluation in the spinal cord

Symptoms: Rapid increase scoliosis due to peripheral n. damage to spinal muscle innerv. Increase weakness & perhaps spasticity in the UE's.

Management - revision of VP shunt; spinal shunting

With increasing scoliosis you can suspect hydromyelia or thethered cord if lesion is below T12. Above T12 ....

Child may not have the musculature to support spine.

Management -

Symmetrical BOS in sit-to-stand, Sitting orthosis or spinal bracing, surgical fusion.

Hip dislocation: most common in high levels due to flaccidity (T12) or L2-3 due to unopposed action of hip flexors and adductors. characteristics?

Varied opinion on whether to surgically intervene as children may lose function.

If not painful, bilateral and pelvis remains symmetrical, surgery may not be indicated.

Convulsive disorders: Occur in __-__%. Happens secondary to __ __ ___ or __; residual injury or brain malformaiton. Management: ____ meds.

10-30%, CSF shunt malfunction or infection. Management: anticonvulsant meds.

Neurogenic bowel and bladder notes:

Fewer than 5% of children develop voluntary control of urinary and anal sphincter.

Training programs can be effectively administered by the time child is 7-8 y.o.

Ind. bowel programs managed by: diet, timing, and laxatives and stimulants.

Bladder: is clean intermittent catheterization effective? What can you do for infection concern? What else can you say about meds and therapy?

Effective. Infection concern: short courses of prophylactic antibiotics.

Meds to supplement program for spastic or hypotonic sphincters are 65% successful.

Variety of injection therapy&surgical alternatives if can not be managed more conservatively.

Increased demineralization due to flaccid paralysis and secondary stimulation from stand is what disease?

Osteoporosis.

studies have not found that standing programs alone, significantly impact the progression of osteoporosis.

Motor paralysis: motor level is the highest intact functional neuromuscular segment, T or F

False. lowest.

International myelodysplasia study group criteria for assigning motor levels that more accurate than traditional neurosegmental levels used for spinal cord injury.

What are the varieties of motor paralysis?

Complete, incomplete, "skip" levels (one or more caudal segments are functioning below last functional level), higher levels may have some degree of spasticity.

Children should be monitored as motor changes are a frequent sign of neurological complication.

What are some sensory losses?

Losses are not always consistent with motor levels.

Sensory impairment impact functional use (big impact)

Integumentary and safety concerns

Should be monitored as frequent sign of neurological complication.

Characteristics of musculoskeletal deformities...

Often have he primary impact on fx

Resulting from muscle fx imbalance & resulting positioning or compensations

impact on positioning, weight bearing, ADL, energy expenditure, mobility.

Focus for prevention and intervention in therapeutic programs.

Musculoskeletal deformities of the extremities and trunk include:

Extremities: position of comfort and in direction of muscle imbalance.

Trunnk: scoliosis, kyphosis, lordosis.

Motor incoordination is secondary to ?

Arnold chiari II - cerebellar ataxia.

Motor cortex or pyramidal tract dysfunction - primary or secondary to hydrocephalus

Motor learning deficits and cognitive dysfunction.

T or F: higher functioning is related to higher level lesions and uncomplicated neurological history.

False - lower level lesions.

Complicated by UE incoordination and memory deficits.

What are some aspects of the Hx part of the examination that should be touched on.

Family and prenatal, postnatal medical, developmental, previous interventions (PT and related support), educational, complaints and concerns that bring child of family for intervention.

What systems could be affected? (Important as associated with additional congenital abnormalities and impact of sensorineural deficits)

Musculoskeletal

neuromuscular

integumentary (skin breakdown; obesity; pt knowledge of skin care routine)

Cariopulmonary (secondary to activity level and musculoskeletal deformities of the spine)

Tests and measures: ROM, joint alignment and integrity can work on...

orthopedic deformities (fixed/flexible motion)

spinal deformities (sitting&standing) - lordosis, kyphosis, scoliosis.

Hip dysplasia, hip anteversion or retroversion

knee deformities -weight bearing and non weight bearing (vargus, valgus, hyperextension)

ROM, joint alignment, and integrity are tests an measures used for....

tibial torsion (atypical muscle pull), ankle and foot deformities; equinus, calcaneal position; longitudinal arch; forefoot adductus; toes (claw, hammer, hyperextended, hallux valgus, plantarflexed 1st ray)

Muscle performance and muscle tonus tests and measures are used for during rationale for thorough assessment....

baseline analysis/progression of impairment

assessing remaining fx/present & future program planning

assess imbalance at each joint relating existing and possible future deformity

Assess need for orthotics or surgery for better alignment/fx

Considerations for evaluations for muscle performance and muscle tonus tests and measures:

every 6 months in first two years; yearly

age of child

cognitive ability

attention span, motivation and ability to follow directions.

What are tonus and UMN signs? (arnold chiari)

Response to passive movement of limbs

CN testing (can change with presence of a shunt)

Coordination

Muscle testing for the UE:

Specific testing of UE muscles

Grip and pinch strenth w/ dynamometer

Muscle testing for LE

Focus on specific muscles

Functional testing (squat to stand, walk on toes)

quantitative measures; dynamometry)

Pressure relief, skin inspection program, compensations for sensory deficit, awareness of sensory deficits and dermatome testing is what testing?

sensory integration during tests and measures

Specific testing involves pin prick or light touch, vibration.

Postural control tests and measures involve what:

Static posture: sitting, standing, sleeping

Balance - trunk control and equilibrium reactions; static, dynamic, anticipatory adjustments.

Peabody, AIMS, GMFM and comprehensive developmental testing is for what test and measures:

Neuromotor performances.

What functional tasks should we look at?

bed mobility, floor mobility, wheelchair mobility and transfers (wheelchair transfers, management, propulsion)

What stuff do we have to work on during gait and locomotion?

Gait deviations, functional analysis on different surfaces, elevations, turning, opening doors

Efficiency and endurance (monitor HR, distance and speed)

Orthotic and assistive devices.

What are some functional and ADL checklists and prerequisite functional tasks we need to work on?

Functional - self care, household management, community level skills

prerequisite - reaching, lifting, carrying.

What do we have to work on with assistive and adaptive devices?

Lifetime management

Appropriateness (specifications and rationale)

Fit and condition

Knowledge of care and management

Monitoring of skin tolerance.

What tests and measure is integrated with muscle tonus to evaluate neurological signs?

Reflex integrity

Test aerobic capacity during:

ADL, Mobility, gait activities.

Tests and measures for ventilation, respiration/circulation

skeletal deformities, trunk and abdominal weakness, increase activity, static positioning, dependency, lack of muscle tonus to support circulation.

Environmenral barriers include:

home, school, and community assessment

Orthotic, patient knowledge of precautions, inspection, and managemewnt of LE is a test and measure for what?

Integumentary integrity

Anthropometric characteristics include:

length, weight, edema (good sign of broken bone, don't complain of pain)

Arousal attention and cognition are tested using:

history, professional observations and developmental testing.

Child's interest and capabilities, family support/lifestyle, resources available and life long feasibility is what test and measure?

Recreational interests

Evaluation, dx, prognosis include what?

Complications of disorder, general health status, multisystem involvement, extent loss of fx, age, resources and support, pt/family goals and desired outcomes.

About this deck

By: Ashley DesJardins
Created: 2012-02-13
Size: 58 flashcards
Views: 40

About StudyBlue

“Simply amazing. The flash cards are smooth, there are many different types of studying tools, and there is a great search engine. I praise you on the awesomeness.”
Dennis