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Cerebral Palsy - General

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Topic updated on 08/12/16 2:40pm
Introduction
  • 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 
  • Epidemiology
    • demographics
      • by definition onset must be before first two years of life
  • 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
  • Associated conditions
    • orthopaedic manifestations characteristic of CP
      • primary
        • abnormal tone
        • loss of motor control
        • impaired balance
      • secondary (growth and spasticity related)
        • spasticity & contractures
        • 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
  • Prognosis
    • most reliable predictor for ability to walk is independent sitting by age 2
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 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 with a low IQ and a high mortality
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; all will eventually be able to walk, regardless of treatment

Gross Motor Function Classification Scale (GMFCS)
Level I  Near normal gross motor function, independent ambulator
Level II Walks independently, but difficulty with uneven surfaces, minimal ability to jump
Level III Walks with assistive devices
Level IV Severely limited walking ability, primary mobility is wheelchair
Level V Nonambulator with global involvment, dependent in all aspects of care

Evaluation
  • History
    • clinical history
      • perinatal history
      • growth & development
      • prior medical treatments
    • functional status  
      • nutritional status
      • respiratory function
      • sitting/standing posture
      • upper and lower extremities function
      • communication skills
      • acuity of hearing and vision
  • Physical Exam
    • musculoskeletal exam
      • motion, tone, and strength
      • hamstring contractures (lead to decreased lumbar lordosis)
      • hip contractures (lead to excessive lumbar lordosis)
    • spine exam
      • presence and flexibility of scoliosis
      • spinal balance and shoulder height
      • pelvic obliquity
Imaging
  • MRI
    • MRI of brain shows a spectrum of changes, with periventricular white matter lesions most frequent (56%), while grey matter lesions (18%)  and brain malformations are less frequent (9%) 
Treatment Spasticity & Deformity
  • Nonoperative
    • physical therapy, bracing/orthotics, and 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
        • 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 
  • Operative
    • soft tissue procedures/releases
      • indications
        • to improve function in child from 3-5 years of age 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 
    • rhizotomy
      • indications
        • ages 4 to 8, ambulatory spastic diplegia, and a stable gait pattern that is limited by lower extremity spasticity
      • technique
        • 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)
    • bony procedures/deformity correction
      • indications
        • usually performed later in life
      • 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

Treatment Fractures
  • Nonoperative

  • Operative
    • ORIF vs. nonoperative depending on fracture pattern

 

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Qbank (7 Questions)

TAG
(OBQ09.171) In patients with cerebral palsy, voluntary control of motion best predicts improvement in function after which of the following? Topic Review Topic

1. Botox injection
2. Selective dorsal rhizotomy
3. Wrist/hand tendon transfer surgery
4. Femoral derotational osteotomies
5. Tibial derotational osteotomies

PREFERRED RESPONSE ▶
TAG
(OBQ09.266) Which of the following decreases acetylcholine levels in the synaptic cleft by blocking the presynaptic release of acetylcholine peripherally? Topic Review Topic

1. Lidocaine
2. Botulinum toxin A
3. Acetylcholinesterase
4. Baclofen
5. Pyridostigmine

PREFERRED RESPONSE ▶
TAG
(OBQ07.12) A six-year-old male child has cerebral palsy and limited ambulatory function. Physical exam is notable for mild spasticity in all four extremities, with the lower extremities demonstrating more involvement than the upper extremities. Cognitive function is mildly delayed for chronologic age. Pelvis radiograph is notable for dysplasia of bilateral hips. Which of the following terms best desribes this patient's cerebral palsy? Topic Review Topic

1. Hemiplegia
2. Diplegia
3. Paraplegia
4. Monoplegia
5. Total body

PREFERRED RESPONSE ▶
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