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Cerebral Palsy

Cerebral Palsy. Health Surveillance Christopher Ewing R1 University of Calgary Pediatrics. What is Cerebral Palsy?. Most common cause of chronic neurological impairment in children Permanent, non-progressive brain injury Motor movement and posture impairments as well as disturbances in:

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Cerebral Palsy

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  1. Cerebral Palsy Health Surveillance Christopher Ewing R1 University of Calgary Pediatrics

  2. What is Cerebral Palsy? • Most common cause of chronic neurological impairment in children • Permanent, non-progressive brain injury • Motor movement and posture impairments as well as disturbances in: • Sensation and perception • Cognition and communication • Gastrointestinal function and nutrition • Social and behavioural • Epilepsy

  3. April 2006 • Cerebral palsy (CP) describes a group of permanent disorders of the development of movement and posture, causing activity limitation, 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 behaviour; by epilepsy, and by secondary musculoskeletal problems.

  4. Epidemiology • Most common cause of childhood disability in Western society. • Incidence approximately 2-3/1000 births • Incidence has not declined despite improvements in perinatal and obstetrical care. • However more premature and VLBW children with severe disabilities are surviving.

  5. Etiology • Often can only be described in 50% of cases • Complex and multifactorial • May result from structural abnormalities of the brain or be acquired • Prenatal/perinatal/postnatal injury due to: • Vascular insufficiency • Toxins • Infections • Prematurity • Birth asphyxia

  6. Differential Diagnosis • Non-progressive • Intellectual disability • Deprivation • Malnutrition • Isolated non-motor and motor handicaps • Spina bifida • Myopathies • Progressive: • Glutaric aciduria type 1 • Arginase deficiency • Sjogren-Larsson syndrome • Metachromic leukodystrophy • Lesch-Nyhan syndrome • Chiari type 1 malformation • Dandy-Walker malformation • Angelman syndrome • Gillespie syndrome • Ataxia-Telangectasia • Hexoaminidase A and B deficiency • Serotendinosus xanthomatosis

  7. Risk Factors • Postnatal (0-2 years) • CNS infection • Hypoxia • Seizures • Coagulopathies • Hyperbilirubinemia • Head trauma • Prenatal • Prematurity • LBW/VLBW • Maternal epilepsy • Hyp(o/er)thyroidism • Infections (TORCH) • 3rd TM bleeding • Drug abuse • Trauma • Multiple pregnancies • Placental insufficiancy • Perinatal • Prolonged labour • Premature ROM • Presentation anomaly • Bleeding prior to labour • Bradycardia • Hypoxia

  8. CNS Pathology • CP lesions often occur in regions sensitive to blood supply disturbance. • Hypoxic-ischemic-encephalopathy (HIE)

  9. Motor • Primary impairments: • Tone (spasticity, dystonia) • Balance • Strength • Loss of selectivity • Sensation • Secondary impairments • Contractures • Deformities • Tertiary impairments (adaptive mechanisms)

  10. Contractures & Deformities • Contractures • Upper extremity: pronator, wrist and finger flexors, thumb adductor • Lower extremity: hip adductor/flexor, knee flexor, ankle plantar flexors • Deformities • Spine: scoliosis, kyphosis • Hip: subluxation, dislocation • Femur/tibia: internal/external torsion • Foot: equinus, valgus, varus

  11. Natural History • Brain lesion is incurable but non-progressive • Clinical picture changes as child grows and develops due to growth and maturation of the CNS and MSK. • Primary movement disorder not evident early on in infancy, although some abnormality may be present • Primitive reflexes persist, and advanced postural reactions fail to develop. • Early intervention can minimize functional effects of neurological impairments.

  12. Early Signs • Many infants appear normal • Abnormal behaviour • Excessive docility/irritability • Poor eye contact or sleep • Oromotor problems • Frequent vomiting • Poor sucking • Tongue retraction or thrusting • Grimacing • Poor mobility • Poor head control • Hand preference, fisting prior to age 2 • Abnormal tone, asymmetric posture

  13. Major Deficits in CP • Loss of selective motor control, and dependence on primitive reflex patterns for movement • Abnormal muscle tone influenced by posture, position, and movement • Imbalance between agonist and antagonist muscles that may lead to contractures and deformities • Impaired body balance mechanisms • Sensory loss

  14. Associated Problems • Seizures • Visual impairments • Intellectual impairment • Learning disabilities • Hearing • Communication • Oromotor dysfunction • Gastrointestinal • Nutrition • Dental • Respiratory dysfunction • Bladder and bowel • Social and emotional

  15. Multidisciplinary Care • Pediatrician • Physiatrist • Developmental pediatrician • Orthopedic surgeon • Neurologist • Speech therapy • Physical therapy • Occupational therapy • Orthotist • Dietician • Ophthalmologist • Audiologist • Teacher • Social Worker • Psychologist

  16. Traditional Classifications • Spastic • Hemiplegic • Diplegic • Quadriplegic • Dyskinetic • Choreoathetoid • Dystonic • Ataxic

  17. Traditional Classifications

  18. Modern Principles of classification • Motor abnormalities • Nature and typology of motor disorder • Functional motor abilities • Associated impairments • Anatomical distribution • Radiological findings • Causation and timing

  19. Health Surveillance • Regular primary and specialized care required to optimize the various and potentially progressive and debilitating health issues in cerebral palsy

  20. History • Developmental milestones • Gross motor function: • head control, sitting, crawling, walking, running • Fine motor function and ADL’s: • feeding, dressing, toilet care, hand function • Speech and sensory • Pain • Social and psychological needs • Caregiver burden, financial and community supports

  21. History • Associated impairment surveillance • Seizure frequency and management • Bowel and bladder function • Communication needs • Oromotor function drooling • Weight gain • Respiratory function • Pressure sores • School function

  22. Physical Examination • Neurological • Mental status, cranial nerves vision, hearing, strength, control, reflexes, tone, involuntary movements, sensory • Musculoskeletal • Range of motion, contractures, deformities, posture • Cardiorespiratory • Abdominal • Skin breakdown

  23. Musculoskeletal Exam • Back • Spinal deformities (scoliosis, kyphosis, lordosis) • Pelvis • Obliquity, stability and sitting balance • Hip • rotation, flexion/adduction contractures • Knee • Patella position, popliteal angle, contractures • Foot and ankle • Gastroc/soleus, tibial torsion, equinus/varus/valgus • Upper extremity • ROM, grasp, pronator contracture/spasticity, function

  24. Functional Examination • Communication • Sitting • Balance • Mobility • Gait • Running, jumping • Hand function • Ability to perform ADL’s

  25. Gait Assessments • Observation • Normal gait • Balance • Propulsion • Video recording • Computerized gait analysis • Kinematics • Dynamic EMG • Kinetics • Energetics

  26. GMFCS • 1. Walks without restrictions • 2. Walks without assistive devices but limitations in community • 3. Walks with assistive devices • 4. Transported or uses powered mobility • 5. Severely limited, dependent on wheelchair

  27. MACS • 1. Handles objects easily and successfully. • 2. Handles most objects but with somewhat reduced quality and/or speed of achievement. • 3. Handles objects with difficulty; needs help to prepare and/or modify activities. • 4. Handles a limited selection of easily managed objects in adapted situations. • 5. Does not handle objects and has severely limited ability to perform even simple actions.

  28. Imaging • X-ray • Cranial ultrasound • Cranial CT • Cranial MRI • EEG

  29. Treatment Goals • Infancy • Optimize physical development, nutrition, and exercise • Childhood • Maximize independent mobility and nutrition • Preschooler • Maximize independent mobility, function, and minimize deformity • Adolescence • Education, vocation and community integration

  30. Treatment Principles • Communication • Activities of daily living • Mobility • Ambulation • Psychosocial

  31. Management Principles • Consider the natural history • Appreciate significance of sensation/perception • Recognize limitation of treatments • Focus on function and comfort, not deformity • Provide functional mobility • Establish appropriate priorities and shift with age • Emphasize child’s assets • Maintain family health • Avoid management fads • Protect the child’s childhood and play experience

  32. Rehabilitation • Physiotherapy • Occupational therapy • Bracing • Assistive devices • Adaptive technology • Sports and recreation • Environment modification

  33. Rehabilitation • Infancy • Educate the family, provide optimal sensorineural development with positioning, stimulation, and exercise. • Childhood • Achieve independent mobility, involvement in self care and ADL’s • School age/adolescence • School integration, social, and vocational skills

  34. Bracing • Increases function • Prevents deformity • Keeps joint in functional position • Stabilize the trunk and extremities • Facilitate selective motor control • Decrease spasticity • Protect extremity from injury in the postoperative phase

  35. AFO • Ankle-Foot Orthotic • Most common brace used in CP • Crucial in spastic hemi/diplegia for mobility • Maintains foot in plantigrade • Provides ground contact and foot clearance • May prevent contractures if worn at night • Hinged, flexible, or solid

  36. Mobility Aids • Transfer aids and lifts • Standers • Walkers • Crutches • Canes • Wheelchairs • Seating system

  37. Spasticity • Part of upper motor neuron syndrome • Increase in physiologic tone as a resistance to passive movement • Velocity dependent • Results in difficulty with movement, posture, and function • May develop into contractures and deformities

  38. Spasticity Treatment • Rehabilitation • Botox injection • Systemic oral medications • Baclofen, benzos, dantrolene, tizanidine, clonidine • Intrathecal baclofen • Surgery

  39. Spasticity Problem Patterns • Hemiplegic • Rectus femoris – stiff knee gait • Gastrocnemius – pes equinus • Arm flexor-pronators – forearm pronation • Wrist/digit flexors - thumb in palm, wrist/digit flexion • Diplegic • Hip adductor-flexors – hip subluxation • Hamstring - knee flexion • Gastrocnemius - pes equinus • Quadriplegic • Hip adductor-flexors – hip subluxation • Hamstring – sacral sitting

  40. Hemiplegic • 20% of spastic CP • Involvement of unilateral arm and leg • Upper extremity involvement > lower extremity • Usually few associated problems, may have seizures, learning or behavioural issues • Common MSK problems: • Forearm pronation, wrist flexion and ulnar deviation, thumb in palm, finger flexion, poor grasp, pes equinus, hemiplegic gait (foot drop, intoeing, knee stiff/flexion)

  41. Diplegic • 50% of spastic CP • Gross motor involvement of lower extremities and minor fine motor involvement of upper. • Usually normal mental function and communication • Primary problem is walking • Common MSK problems: • Hip flexion/IR/adduction and scissoring, ankle equinus, jump/crouch gait, risk of hip instability

  42. Quadriplegic • 30% of spastic CP • Involvement of neck, trunk, and all four extremities • Severe motor impairment and other signs and symptoms of CNS dysfunction • Cognitive, seizures, speech and swallowing • Frequent comorbid medical associations • Higher mortality • Common MSK problems • Hip dislocation, spinal and postural problems

  43. Dyskinetic • Hyperkinetic/Choreoathetoid • Purposeless, often massive involuntary movements • Poor selective motor control/coordination • Dystonic • Abnormal shifts in muscle tone in stereotyped pattern • MSK issues • Ambulation dependent on severity, clumsy and unsteady gait, motor planning and execution, scoliosis, degenerative hip disease

  44. Spine • Scoliosis • Most common spinal deformity in CP • Progressive, causes difficulty with sitting and respiratory function • Related to severity of CP neuromuscular involvement • Hyperlordosis • Hyperkyphosis • Pelvic obliquity

  45. Hip Surveillance • Hip dislocation is a common concern in cerebral palsy and can be debilitating • Second most common MSK deformity in children (second to equinus) • Directly related to GMFCS • Common in spastic CP • Progressive instability due to: • Muscle imbalance, persistent primitive reflexes, faulty posture, absence of weight bearing.

  46. Hip Surveillance

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