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Updated: Oct 23 2021

Cerebral Palsy - General

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  • summary
    • Cerebral Palsy is a common congenital condition caused by injury to the immature brain that leads to upper motor neuron disease and presents with cognitive and musculoskeletal manifestations of varying severity.
    • Diagnosis is made clinically with evaluation of developmental milestones, cognitive function, and musculoskeletal abnormalities including spasticity, loss of motor control, and impaired balance.
    • Treatment involves a multidisciplinary approach to address spasticity, orthopedic manifestations, and cognitive function. 
  • Epidemiology
    • Incidence
      • 2-3 per 1000
    • Demographics
      • by definition onset must be before first two years of life, although diagnosis may be delayed in very mild cases
      • most common cause of chronic childhood disability
  • Etiology
    • Nonprogressive upper motor neuron disease (static encephalopathy) due to injury to immature brain
      • orthopaedic manifestations
        • contractures (this topic)
        • fractures (this topic)
        • upper extremity deformities
        • hip subluxation and dislocation
        • spinal deformity
        • foot deformities
        • gait disorders
    • Pathophysiology
      • pathoanatomy
        • leads to muscle imbalance with a mixture of weakness and spasticity
        • the encephalopathy is static while the affected portion of the musculoskeletal system changes with growth
      • risk factors
        • prematurity (most common)
        • anoxic injuries
        • prenatal intrauterine factors
        • perinatal infections
          • toxoplasmosis
          • rubella
          • cytomegalovirus infection
          • herpes simplex
          • ToRCH
        • meningitis
        • brain malformations
        • brain trauma - NAT
    • Associated conditions
      • orthopaedic manifestations characteristic of CP
        • primary
          • abnormal tone
          • loss of motor control
          • impaired balance
          • spasticity
          • (hypotonia)
          • (dyskenesias such as chorea and athetosis)
        • secondary (growth and spasticity related)
          • contractures
            • starts as dynamic contractures, become static with time (continuous muscle contraction results in shortening) and growth (growth of bones occurs at a faster longitudinal rate than muscles in spastic cerebral palsy)
          • upper extremity deformities
          • hip subluxation and dislocation
          • spinal deformity
          • foot deformities
          • gait disorders
          • fractures
            • often associated with non-ambulators secondary to low bone mineral density
            • bisphosphonates may be useful
              • IV pamidronate considered with >3 fractures and a DEXA z-score <2 SD
  • Classification
      • Physiologic Classification
      • Spastic
      • Most common
      • Velocity-dependent increased muscle tone and hyperreflexia with slow, restricted movement due to simultaneous contraction of agonist and antagonist muscles.
      •  Most amenable to operative treatments.
      • Athetoid
      • Characterized by a constant succession of slow, writhing, involuntary movements
      • Ataxic
      • Characterized by inability to coordinate muscle movements.
      • Results in unbalanced, wide based gait.
      • Mixed
      • Usually mixed spastic and athetoid features and involves the entire body
      • Hypotonic
      • Usually precedes spastic or ataxic for 2-3 years
      • Anatomic Classification
      • Quadriplegic
      • Total body involvement and nonambulatory
      • Diplegic
      • Legs more than arms but usually still ambulatory.
      •  IQ may be normal (injury in brain is midline) 
      • Hemiplegic
      • Arms and legs on one side of the body, usually with spasticity
      • Will eventually be able to walk, regardless of treatment
      • Gross Motor Function Classification Scale (GMFCS)
      • Type I
      • Near normal gross motor function, independent ambulator
      • Type II
      • Walks independently, but difficulty with uneven surfaces, minimal ability to jump
      • Type III
      • Walks with assistive devices
      • Type IV
      • Severely limited walking ability, primary mobility is wheelchair
      • Type V
      • Nonambulator with global involvment, dependent in all aspects of care
  • Evaluation
    • History
      • clinical history
        • perinatal history
        • growth & development
        • prior medical treatments
      • functional status
        • sitting/standing posture
        • upper and lower extremities function
        • communication skills
        • acuity of hearing and vision
    • Physical exam
      • general musculoskeletal exam
        • motion, tone, and strength
        • Rotational limb profiles for torsional deformities
      • gait
        • gait lab analysis
        • plantigrade feet
        • crouch
        • stiff knee gait
      • spine exam
        • presence and flexibility of scoliosis
        • spinal balance and shoulder height
        • pelvic obliquity
        • resting head posture
        • hamstring contractures (lead to decreased lumbar lordosis)
      • hips
        • hip contractures
          • flexion contracture (lead to excessive lumbar lordosis)
          • adduction contracture
        • hip instability and dislocations are common, may be looked over as a contracture alone
          • observe thigh length in sitting, leg length when supine, or galleazzi test
          • adductor contracture can make examination difficult
      • foot and ankle
        • equinovarus and planovalgus deformities common
        • observe wear patterns, callouses
        • note hypertonicity
        • toe walking or absent heel strike during gait secondary to gastrosoleus spasticity and contracture
          • may hyperextend knee to obtain heel contact
        • provacative Silverskiold test to differentiate gastrocnemius contracture vs achilles contracture
  • Imaging
    • Radiographs
      • standard radiographs should include
        • AP and lateral of hips
        • standing spine radiographs as baseline
    • MRI
      • MRI of brain shows a spectrum of changes including
        • periventricular leukomalacia (PVL) white matter lesions most frequent (56%)
        • while grey matter lesions (18%)
        • brain malformations are less frequent (9%)
  • Treatment Spasticity & Deformity
    • Nonoperative
      • physical therapy, bracing/orthotics, medications for spasticity
        • spasticity control
          • Botox (botulinum - A toxin)
            • competitive inhibitor of presynaptic cholinergic receptors with a finite lifetime (usually lasts 2-3 months)
            • used to maintain joint motion during rapid growth when a child is too young for surgery
            • often injected into gastrocnemius
            • helpful treatment in dynamic contractures; little benefit with static contractures
          • baclofen
            • reduces tone via unknown mechanism
              • thought to act as GABA agonist
              • intra-thecal administration is preferred route to avoid cognitive impairment seen with oral administration (poor bioavailability leads to difficulty with oral dosing)
              • Intra-thecal baclofen tends to be used for non-ambulatory CP (GMFCS IV and V)
            • side effects
              • baclofen withdrawal
                • can cause severe fevers, hyperkalemia, and elevated CPK levels
    • Operative
      • soft tissue procedures/releases
        • indications
          • to improve function in child from 3-5 years of age with spasticity and voluntary muscle control
        • techniques
          • tenotomies for continuously active muscles (e.g. hip adductor)
          • tendon lengthening for continuously active muscles (e.g. achilles tendon or hamstring)
          • tendon transfers for muscles firing out of phase (e.g. rectus tendon or tibialis posterior)
            • tendon transfers in the upper extremity show the best improvement in function in patients with voluntary motor control
      • selective dorsal rhizotomy
        • indications
          • ages 4 to 8, ambulatory spastic diplegia, and a stable gait pattern that is limited by lower extremity spasticity
        • neurosurgical resection of dorsal rootlets that do not show a myographic or clinical response to stimulation
        • contraindications
          • athetoid CP
          • nonambulatory patients with spastic quadriplegia (associated with significant spinal deformities)
        • falling out of favor due to limited functional gains and no reduced risk of subsequent musculoskeletal surgeries
      • bony procedures/deformity correction
        • indications
          • usually performed in later childhood / adolescence
          • static contractures, progressive joint breakdown, and certain patterned gait-deterioration can be treated with combinations of myotendonous unit lengthening, tendon transfers, and osteotomies
        • SEMLS surgery (Single-Event, Multi-Level Surgery)
          • concept arose to limit multiple surgeries, anesthetics, and rehabilitation time for children
          • most successful when combined with a thorough gait lab assessment that predicts improvemenet in function with multiple level surgical interventions
          • simple lengthenings can cause deterioration in gait when other contractures are "uncovered"; SEMLS management seeks to avoid these iatrogenic complications
          • can be done on bilateral lower extremities in efforts to improve gait
        • specific procedures
          • see Cerebral Palsy Upper Extremity Conditions
          • see Cerebral Palsy Spine Conditions
          • see Cerebral Palsy Hip Conditions
          • see Cerebral Palsy Gait Disorders
          • see Cerebral Palsy Foot Disorders
  • Prognosis
    • Most reliable predictor for ability to walk is independent sitting by age 2
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