Cerebral Palsy

See Also

Definitions

Cerebral Palsy
1. Non-progressive, permanent Developmental Disorder of childhood due to brain injury or malformation
2. Results in impaired Muscle coordination and movement

Epidemiology

Incidence: Up to 2.5 per 1000 U.S. born children
1. Most common physical Disability in children

Pathophysiology

Brain injury occurring before age 3 to 5 years
Results in non-progressive disorder of movement and Posture
Wide variability in disease involvement and in degree of intellectual capacity

Causes

Idiopathic in 25% of cases (up to 80% in some reviews)
Prenatal causes or risk factors: Up to 80-90% of cases
1. Delivery complications (i.e. asphyxia): 6% of cases
2. Preterm birth before 32 weeks or <2500 grams
3. Intrauterine Growth Retardation
4. Intracranial Hemorrhage
5. Teratogen Exposure
6. Perinatal infection (Chorioamnionitis)
7. Multiple Gestation
Postnatal causes or risk factors (<8 to 20%)

Types

Limb spasticity (80% of cases)
1. Features
  1. Hypertonic Muscle movement
  2. Hyperreflexia
  3. Scissors gait
  4. Toe-walking
2. Motor Impairment Categories
  1. Diplegia
  2. Hemiplegia
  3. Quadriplegia
Dyskinesia (10-20% of cases)
1. Slow, writhing, uncontrollable limb movements worse during stress
Ataxic Cerebral Palsy (5-10% of cases)
1. Wide-based gait
2. Intention Tremor
Mixed Cerebral Palsy
1. Combination or other features

Associated Conditions

Coordination and Movement Disorder
1. Features vary depending on Cerebral Palsy type (see above)
2. Typically limb spasticity, Dyskinesia or Ataxia
Sensory deficits
1. Altered sensory Perception (pain on light touch)
Chronic Pain (75%)
Cognitive Impairment (50-66% of cases)
Seizure Disorder (25 to 50-66% of cases, often spastic type)
Inability to walk (33%)
Inability to speak (25%)
Growth Delay
Hearing Impairment
Vision Impairment
1. Screen for Strabismus and Hemianopia
Gastrointestinal disorders
1. Vomiting (Delayed Gastric Emptying)
2. Constipation (GI motility, Dehydration, mobility)
3. Risk of Sigmoid Volvulus
Swallowing difficulty
1. Aspiration risk
2. Drooling
3. Inadequate oral intake
Osteoporosis (90%)
Hip displacement (33%)
Urinary Incontinence (25%)
Behavioral or emotional disorders
Sleep Disorder

Diagnosis

Systematic, stepwise approach to diagnosis
1. Thorough history and physical
2. Recognize permanent, non-progressive motor function disorder
3. Evaluate for comorbidities associated with Cerebral Palsy
4. Obtain imaging if perinatal Ultrasound is not sufficient for diagnosis (see below)
5. Consider differential diagnosis (see below)
Findings suggestive of Cerebral Palsy
1. Slow motor development
2. Altered Muscle tone and Posture
3. Moro Reflex persists beyond 6 months of age
4. Dominant hand preference established under 12 months
Findings of alternative diagnosis (neurodegenerative)
1. Loss of acquired skills
2. Atypical body odor (as seen in metabolic disorders)
3. Loss of Deep Tendon Reflexes
Severity Assessment Tools
1. Gross Motor Function Classification System (CMFCS) for Cerebral Palsy
  1. Age based tool that rates mobility, Posture, and balance each on 5 point scale (1 for mild, 5 for severe)
  2. https://cerebralpalsy.org.au/our-research/about-cerebral-palsy/what-is-cerebral-palsy/severity-of-cerebral-palsy/gross-motor-function-classification-system/
  3. Palisano (1997) Dev Med Child Neurol 39:214-23 [PubMed]
Timing of diagnosis
1. Cerebral Palsy may be suspected on perinatal Ultrasound or postnatal Brain MRI <6 months
2. Diagnosis may not be made until age 12-24 months in areas with fewer Imaging Resources
Resources
1. Movement Disorder examples
  1. https://www.youtube.com/watch?v=cOfUGUNxEqU

Imaging

Perinatal Ultrasound (Fetal Survey)
Newborn transcranial Ultrasound
Brain MRI (variable findings depending on cause)
1. Schizocephaly (cerebral tissue clefts)
2. Hydrocephalus
3. Periventricular leukomalacia

Differential diagnosis

Consider neurodegenerative disorders and Inborn Errors of Metabolism
Examples
1. Arginase deficiency
2. Glutaric aciduria
3. Niemann-Pick Disease
4. Lesch-Nyhan Syndrome
5. Rett Syndrome

Management

Approach
1. Involve a multispecialty care team (surgery, OT, PT, speech-language, social work, psychology)
2. Assistive Devices for Activities of Daily Living
3. Enable mobility, optimize functionality and independence
4. Address Mood Disorders
5. Establish realistic goals with family
  1. Expect nearly best motor development by age 5 years
  2. Goals change over time (e.g. communication, social, academic)
Global therapies with variable efficacy
1. Neurodevelopmental treatment (Bobath method)
2. Conductive education with rehabilitation program
Communication
1. Speech therapy may aid communication (e.g. voice synthesizers)
Hearing
1. Screen Hearing every 6 months from age 6 months to 3 years
Vision
1. Screen Visual Acuity at 1 and 4 years
2. Observe for Strabismus and Hemianopia
Physical therapy
1. Balance benefit with stress of frequent visits
2. Improve hand function
  1. Constraint induced movement therapy
    1. Dominant hand constrained to promote use of nondominant hands
    2. May be more frustrating technique for children
  2. Hand-arm intensive bimanual therapy
    1. Also encourages use of both hands
3. Resistive Exercise is controversial
  1. Has been avoided due to increased spasticity risk
  2. Recent studies suggest strengthening is beneficial
    1. Dodd (2002) Arch Phys Med Rehabil 83:1157-64 [PubMed]
Medications: Spasticity
1. Botulinum Toxin (Botox) injections for leg spasticity
  1. Reddigough (2002) Dev Med Child Neurol 44:820-7 [PubMed]
2. Baclofen (Lioresal) intrathecal
  1. See Baclofen Pump
  2. Campbell (2002) Dev Med Child Neurol 44:660-5 [PubMed]
Medications: General
1. Pain (especially Hip Pain)
  1. Assess pain and treat adequately
  2. Hip Dislocation may occur spontaneously due to spasticity
2. Constipation
  1. Bowel regimen (fluids, fiber, Stool Softeners)
3. Osteoporosis
  1. Consider DEXA Scan (adults) and Osteoporosis treatment to prevent Fractures
Seizures
1. Emergent Seizure management is the same as for non-Cerebral Palsy patients
  1. Administer Benzodiazepines and Anticonvulsant loading
  2. Check Serum Glucose and anticonvulsant levels
  3. Other labs and diagnostic evaluation only as indicated by exam and history
2. Seizures may be refractory to standard measures
  1. Combination antiepileptics are often required for Seizure control
  2. Consider Ketogenic Diet (risk of Drug Interactions)
Surgery
1. Leg spasticity
  1. Selective dorsal rhizotomy
    1. Selective cutting of L1-S2 dorsal rootlets
2. Muscle imbalance with hip subluxation, dislocation
  1. Abduction bracing
  2. Soft tissue release
  3. Femoral or pelvic osteotomy
3. Implantable stimulator to superior-medial Cerebellum
  1. Davis (2000) Arch Med Res 31:290-9 [PubMed]
4. Gastrostomy for Swallowing and eating difficulties
  1. Samson-Fang (2003) Dev Med Child Neurol 45:415-26 [PubMed]
Urinary Incontinence
1. Consider physical therapy, biofeedback and medications
2. Consider Bladder stimulators (neuromodulation)
Prevent Pressure Ulcers
1. Optimize repositioning and support surfaces
2. Early wound care Consultation for Pressure Sores
Adjuncts
1. Lower limb orthoses (variable evidence)
  1. Ankle Orthotics to decrease equinus deformity and improve dorsiflexion
2. Elastic body suits (difficult compliance)
3. Mittens (due to sensory disorder)
  1. Prevent injury to fingers and hands, esp during certain periods such as Teething

Prevention
Secondary conditions

Observe for cancers of higher risk in Cerebral Palsy
1. Brain cancer
2. Breast Cancer
3. Routine Health Maintenance
  1. Left lateral position more comfortable for pelvic
Educate about injury risks
1. Drowning risk
2. Motor Vehicle Accidents

References