Pediatrics > Cerebral Palsy > Flashcards

Cerebral Palsy Flashcards

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1
Q

CP Defenition

A
  • Cerebral palsy (CP) describes a group of permanent disorders of the development of movement and posture causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.
  • The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems
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2
Q

Etiology - Birthweight

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  • Birthweight:
    • 1000-1499 grams (2.2-3.3 lbs) highest prevalence of CP
    • Below 1000 grams had no more increased risk than 1000-1499 grams
    • Lowest risk if birthweight >2500 grams (5.5 lbs) live births
  • LBW: infants – periventricular leukomalacia (PVL,) periventricular hemorrhage, cerebral infarct
  • Normal weight term infants – hypoxic-ischemic encephalopathy (HIE) most common cause (resulting in dyskinetic or spastic quad CP
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3
Q

Etiology – Gestational Age

A
  • Gestational age:
    • Highest prevalence of CP at 23 weeks GA, lowest after 36 weeks GA
    • Biggest risk if born <28 week
    • Term infants: ~55-65% of kids with CP were term
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4
Q

Genetics

A
  • Genetics: some evidence there are phenotypes for increased risk for CP
  • Already identified 6 genes
  • Single-gene mutations found in some cases
  • Familial cases
  • 2-5 times more common in consanguinity
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5
Q

PVL

A
  • Often caused by HIE
  • Associated with cognitive impairments
  • PVL: >80% develop CP
  • Posterior lesions worse than anterior lesions
  • Focal cysts associated with spastic diplegia
  • Extensive cysts associated with spastic quadriplia
  • Non-cystic PVL also associated with CP
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6
Q

PVL/ IVH

A
  • Grades I-II PVL likely to walk by 2 yo
  • Grades III-IV, only 10% walk
  • Can also have intraventricular hemorrhage (IVH)
  • Grades I-II, minimal risk of neurological impairment
  • Grades III-IV, high risk of neurological impairment
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7
Q

Maternal risk factors

A
  • Seizure disorder
  • Thyroid disease
  • Cognitive impairment
  • Heart disease
  • Respiratory disease
  • Hypertension
  • >40 yo
  • Pre-eclampsia
  • Chorioamnionitis
  • Still birth or neonatal death
  • Abnormal amount of amniotic fluid
  • Bleeding in 2nd or 3rd trimester
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8
Q

CP: Early Diagnosis

A
  • Preemie with abnormal MRI and abnormal motor signs on General Movements or Test of Infant Motor Performance useful in identifying the presence and location of an injury in ∼89% of kids with CP
  • Doesn’t predict the severity of CP
    • (AIMS/TIMP in 4-10 month old for prediction of and ID of CP)
  • 25-50% of babies with CP will not show signs of CP as newborns
  • Prechtl’s qualitative assessment of general movements (GMs) is the most predictive assessment tool to detect infants, as young as 3 months who have the highest risk of CP…”
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9
Q

General Prognosis

A
  • “All children with cerebral palsy will have physical challenges. The bigger the child’s brain injury, the more likely the child is to have other co-occurring impairments, diseases, and functional limitations accompanying the physical disability, except for pain and behavior, which are common regardless of the level of physical disability.”
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10
Q

Predictors of Independent Walking in Young Children with CP

A
  • Sit-to-stand, and stand to sit
    • Based on the postural control
    • and functional strength needed
  • Closed chain exercises mimicking everyday activities has shown to increase strength and functional ability
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11
Q

Visual Deficits

A
  • Up to 71%
  • ROP
  • Nystagmus
  • Homonymous hemianopsia (25% of hemiplegia)
  • Strabismus
    • Esotropia: deviation of eye towards midline (more common)
      Exotropia: deviation of eye away from midline
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12
Q

Modified Ashworth: Body Structure/Function-Spasticity

(Quantifying Muscle Tone)

A
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13
Q

Classification

A
  • Anatomic distribution and location
    • Diplegia: both lower extremities
    • Hemiplegia: UE &LE on one side of body
    • Quadriplegia: all 4 extremities
    • Trunk can be involved in all
  • Movement Disorders
  • Gross Motor Function Classification System
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14
Q

Movement Disorders

A
  • Related to location of brain damage
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15
Q

Spastic CP

A
  • Spastic: motor cortex or white matter projections to/from cortical sensorimotor
    • Produces abnormal patterns of posture and movement
  • Increased muscle tone in antigravity muscles
  • Abnormal postures and movements with patterns of all flexion or all extension
  • Imbalance of tone across jointsà contractures and deformities (hip flexors, adductors, internal rotators; knee flexors; ankle plantarflexors; scapular retractors; glenohumeral extensors and adductors; elbow flexors; forearm pronators
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16
Q

Dyskinetic forms:

A
  • Dyskinetic forms (uncontrolled and involuntary movement); basal ganglia involvement
    • Results in general instability, abnormal postural patterns, lack of coordinated, rhythmical, and accurate movements
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17
Q

Dyskinetic movements can be:

A
  • Twisting and repetitive movements – known as dystonia
  • Slow, ‘stormy’ movements – known as athetosis
  • Dance-like irregular, unpredictable movements – known as chorea.
  • Dyskinetic movements often co-occur alongside spasticity.
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18
Q

Dystonia

A
  • “Dystonia is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both.” (Sanger et al, 2010, p. 1541)
    • More predictable movements than chorea
    • Triggered by voluntary attempt to move
    • Absent during sleep
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19
Q

Chorea

A
  • “Chorea is an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments.”
  • Movements are brief, jerky, discrete motions appear to constantly move
  • “…multiple, repeated, but not rhythmic movements”
  • Motions more rapid than dystonia and worsen with movement
  • Doesn’t stop with relaxation
  • Caused by damage to the cerebral cortex, basal ganglia, cerebellum, or thalamus
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20
Q

Ballism

A
  • “…ballism as chorea that affects proximal joints such as shoulder or hip. This leads to large amplitude movements of the limbs, sometimes with a flinging or flailing quality.”
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21
Q

Athetosis

A
  • “Athetosis is a slow, continuous, involuntary writhing movement that prevents maintenance of a stable posture.”
  • Smooth, “sinuous, continuously…flowing, ongoing, random movement…”
  • Snake-like movements
  • Same regions of body vs. chorea
  • Present at rest as well as when attempting to move
  • No sustained movement as in dystonia
  • Distal more than proximal; trunk, face, neck
  • Use weights distally, weighted belt, weighted vest, weighted walker
  • Use approximation
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22
Q

Athetoid CP (Dyskinetic)

A
  • Decreased muscle tone; floppy baby
  • Poor proximal joint stabilityà poor functional stability
  • Decreased coordination when child assumes upright
  • Poor visual tracking
  • Speech delay and oral motor problems/drool
  • Tonic (primitive)reflexes persist interfering with functional posture and movement
    • (so use sidelying positions to decrease effect
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23
Q

Ataxia

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A
  • Ataxia: Inability to make smooth, accurate, coordinated movements
  • Multiple causes including CP - results from damage to cerebellum
  • (also genetic disorders, CNS damage, fetal alcohol syndrome)
  • “…abnormal movements in ataxia increase near a target (intention tremor and dysmetria) and improve with stabilization of proximal joints…”
24
Q

Ataxic CP

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A
  • Low postural tone à poor balance
  • WBOS; unsteady/staggered gait
  • Uncoordinated movement
  • Initial hypotonia followed by ataxia
  • Intention tremor of hands
  • Poor visual tracking, nystagmus
  • Speech articulation problems

Use deep pressure/approximation

Lycra suit to improve proximal stability

Weighted walker (Increase proprioception)

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Gross Motor Function Classification Scale (GMFCS)
* Level I most functional to Level V most involved * Provides method of predicting child’s outcome * Maximal mobility generally between 9-12 yo * Overview of child’s current functional abilities * Based on performance in daily life * self initiated movement with emphasis on sitting and walking * functional limitations * Need for handheld AD * Need for wheeled mobility * quality of movement (somewhat) * **NOT** an outcome measure: *Do not* set goal of treatment to change someone’s classification
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ICF: Body functions and structure
* Muscle Tone Abnormality * Muscle Strength: Insufficient force generation * Skeletal Structure Changes: * Torsion of long bones * Joint instability * Premature degenerative changes in WB jts * Boney malalignment (scoliosis) * Hip subluxation/Dislocation * fractures
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Best predictor of ambulation
* Sitting independently by 24 months * (walk at least 15 meters w/ or w/o AD by 8yo) * If not sitting independently by 36 months, low chance of achieving a functional independent ambulation
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PT intervention: Spasticity
* Characterized primarily by 2 patterns * Croucher * Extender \**Cognitive, visual, auditory and oral motor deficits*
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Spastic CP-*Croucher*
* Think about the posture of a child who has a crouched gait (eg excessive knee flexion). * Think about what muscles need to be strengthened for a child who has a crouched gait?
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Crouched gait
* Posture * Hip flexion * Hip adduction * Hip IR * Knee flexion * Genu valgum * Foot pronation * Excessive ankle dorsiflexion * WBOS----strong influence on hip (address it) * (also need a vertical tibia for line of gravity to go through joint to keep hip and knee straighter) * Use orthotic to control foot if needed to get above
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Crouched gait muscle effects
* Tight fascia lata * Poor glut max * Tight hamstrings * Tight hip adductions -can be consequence of tight TFL; if TFL tight then if you adduct hip, the leg will move into hip IR and flexion (supine and stand) Doing adductor release will not help!
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Croucher
* Glut max is key to hip and trunk control-must get active hip extension to get long term carry over to correct hip IR also * Stabilizaiton of pelvis by abdominals and elongation of hips flexors * Hip extensors and abdominal muscles act as force couple to posteriorly tilt pelvis * If cannot extend hip joint then extension comes in the low back leading to increased lumbar lordosis * So must elongate hip flexors while also trying to activate abdominals and glut max * Work in half kneel-straddle bolster and rotate to each side into half kneel (can then move body to stretch hamstrings or rectus femoris) * Tight hip flexors→ anterior pelvic tilt * Tight hip extensors→ posterior pelvic tilt * If hip joint is restricted into flexion because of a posterior pelvic tilt/tight hip extensors * lumbar extension becomes lumbar flexion * No mobility at hips in pull to sit * Rectus abdodminus used for stability (not mobility) * This further increases the posterior pelvic tilt and hip extension
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Interventions
* Think about the posture of a child who has an “extender” gait. * Think about what muscles need to be strengthened for a child who has an extender gait? * Besides strengthening, how else do we treat the impairments?
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Extender
* High tone and strong extension-cannot move out of sagittal plane * Look the same in standing as they do in horizontal * No lower extremity dissociation * Try to work with arms forward to decrease scapular adduction * Hip muscles not working-quads work overtime extending knee and stretching hamstrings and gastroc (hamstring stretch mechanically extends hip and gastroc stretch mechanically plantar flexes ankle
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Quads
* Stretch of tight hamstrings at distal attachment (knee) causes proximal attachment to move posteriorly into posterior pelvic tile (hip extension) * Knee extension also stretches the gastrocnemius muscle and cause plantar flexion ## Footnote *\*Quads used to compensate for weak glut max*
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PT Intervention: Hypertonia
**Facilitate normal motor milestones:** ◦Prone on elbows ◦Rolling ◦Sitting ◦Standing ◦Commando crawling on belly ◦Transitioning into/out of sitting and quadruped ◦Creeping on hands and knees ◦Pulling to standing ◦Lowering self to sitting ◦Cruising ◦Standing independently ◦Ambulating using push cart ◦Ambulating with 1-2 hands held ◦Taking steps independently ◦Ambulating independently
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PT Intervention- Infancy
* Everyday handling and positioning of infants influences development * Don’t forget importance of mid-line activities and promotion of symmetry * Include all planes of motion in movement act * A/Pà lateral à rotational * Self exploration * Promote environmental and social interaction * Trial and error * Start standers if possible
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PT Intervention-Preschool
* Development of locomotor, cognitive, communication, fine motor, self-care and social abilities * Control of posture/alignment and mvts * Look for limits in participation * Muscles need to be extended to their limits regularly * Bones need compressive forces * Cardiovascular system challenged * STANDERS * **Main goal is for independent mobility**
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Upright Mobility
Posterior walkers/posture walkers * Encourages upright posture during gait * Promotes improved gait characteristics * Decreases energy expenditure compared to anterior walker GMFCS Level IV can use wheeled walking devices that support trunk and pelvis * allows for interaction with environment/participation
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PT Intervention-School-Age/and up
* More of the same but focus of child may shift to school and community life * TM training with PBWS (GMFCS III-V) * Aquatics * Prevention of secondary impairments * Maintenance of achieved level of motor function as child enters adolescence * Prevention of overuse syndromes * Progressive resistance training recommended * Therabands * Free weights * Isokinetics * Functional movements
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Strength (Muscle Force Production)
* Strength can be improved via free weights, isometric, isokinetic, open and closed-chain isotonic, body weight, weighted backpacks, pulley systems, and electrical stimulation but its impact on activities or participation unknown * Effect of strengthening on gait, gross motor function, self-esteem, or quality of life inconclusive and/or limited * At least 3/week for 6 weeks
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Bone Mineral Density
* *Skeletal loading interventions*: effects on kids with CP inconclusive – more effect on kids who are ambulatory * *Type of loading important*: static activities, vibrating platforms, standers, combination of weight-bearing and strengthening activities * 30 minutes, 5/week x 2 mo or 2/day, 30 minutes x6 mo * Amount of force needed not documented * May need longer duration in kids with CP * *Medications/vitamins*: growth hormone, calcium, vitamin D, bisphosphonates * Unknown what type is better in child to prevent osteoporosis as adult with CP * Unknown impact on activity or participation
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Locomotor training
* Affects gait speed, endurance, community ambulation * Studies have used variety of self-paced, pre-set pace, fasted pace, and variety of amount of body weight support * 30 minutes 2/day x2 weeks or 2/day x6+weeks
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Interventions: Evidence Present
* Ankle serial casting (3-6 weeks) * Orthotics-AFOs improve gait kinematics * Static vs. dynamic AFOs * Fitness training * Hip monitoring * Prevention of pressure sores * CIMT * Context-focused therapy * Goal directed/functional training * HEP .
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Mobility Opportunities via Education (MOVE)
* 4 components * Sitting * Standing * Walking * Transitioning \*Based on the principle that Movement is the foundation of all learning
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Goal, Activity, Motor, Enrichment | (GAME)
* 3 components: * 1.goal oriented activity-based motor training * 2.parent education * 3.ways to enrich the child’s learning environment \*The motor learning component of the intervention is based on the principles of motor learning and dynamic systems theory
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UE Training
* CIMT: can affect body structure and function, activity, and participation levels * More practice is better (practice based/goal directed) * 90 hours, 15 days better than 60 hours, 10 days
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Participation level
* Community fitness programs * Powered child cars
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Medical interventions
* Medications * Orthopedic surgery * Neurosurgery
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Meds- Management of Spasticity
* Oral meds-presynaptic inhibition of acetylcholine release * **Baclofen** ---CNS * Diazepam (valium)---CNS * Tizanidine (zanaflex) ---CNS * Dantrolene sodium (dantrium) ---muscle * **Baclofen pump-intrathecal programmable pump** * **GABA b-agonist (Inhibitory CNS neurotransimitter)** -Catheter delivers drug to subarachnoid space in SCà ms. relaxation (controls spasticity below SC level to which it is delivered)
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Baclofen pump
* Pros * Longer lasting, more specific site of action, fewer side effects, can hold up to 4 month supply * Cons * Side effects of hypotonia, nausea, HA, 2° issues at surgical site or equipment failure/kinked catheter, overdose and withdrawal possible * Overdose * Very drowsy, dizzy, respiratory depression, seizure, hypotonia, loss of consciousness (call 911) * Withdrawal * Increase spasticity, tingling, HA, hyperthermia, hypotension, seizures, hallucinations, altered mental state, autonomic dysreflexia (call 911), itching without rash MOST COMMON IN KIDS WITH SPASTIC QUADRIPLEGIA
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Injectible Meds
* *decrease* local spasticity & Dystonia * decrease contractures * *increase* motor control * Non-systemic * *Botox* * Interferes with release of acetylcholine at NMJ * Directly into affected muscles-No systemic effect * Lasts 3-6 months (expensive) * Important to combine with casting/orthotics/night splinting, positioning, stretching and motor strengthening * Anesthetics/Diagnostic Nerve Blocks (Lidocaine) * Neurolyitc Nerve blocks (ethanol/phenol)
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Orthopedic Surgeries- Soft tissue related
* Decrease contractures * Decrease abnormal bony alignment * Improve motor control and function * Soft tissue related * Tendon (tenotomy) or muscle lengthening * Most common: achilles tendon, hamstrings, iliopsoas, hip adductors, and hamstrings * Tendon/muscle transfers * Attachments moved to change direction of force * Most common: hip adductors transferred to hip abductor
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Orthopedic surgeries- Bone related
* Bone-related * Cut, remove or reposition bone to facilitate normal alignment and prevent subluxation or dislocation * Osteotomies * Most common: femoral, tibial or pelvic * Femoral derotation osteotomy * Fusions * Spinal fusions -Non-weight bearing after bony procedures and risk of weakness and decreased function
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Neurosurgery
* Neurectomy, selective dorsal rhizotomy (SDR), deep brain stimulation * SDR * Typically between ages of 4-10yo (& GMFCS I-III) * Sever the dorsal sensory nerve roots with abnormal responses to stimulation * Decreases spasticity à improved motor control * Possible sensory loss and anesthesia/surgery risks * Not reversible * *Ambulation is always the goal: intensive strengthening program required following surgery* -Most common in kids with spastic diplegia with selective muscle control and no deformity, good motivation
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Adeli Suit
* Originally designed by the Russian space program for cosmonauts to maintain muscle tone in a weightless environment, the Adeli Suit has been modified to help people with cerebral palsy improve gross motor function and gait
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