II. Definitions
- Cerebral Palsy
- Non-progressive, permanent Developmental Disorder of childhood due to brain injury or malformation
- Results in impaired Muscle coordination and movement
III. Epidemiology
-
Incidence: Up to 2.5 per 1000 U.S. born children
- Most common physical Disability in children
IV. 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
V. Causes
- Idiopathic in 25% of cases (up to 80% in some reviews)
- Prenatal causes or risk factors: Up to 80-90% of cases
- Delivery complications (i.e. asphyxia): 6% of cases
- Preterm birth before 32 weeks or <2500 grams
- Intrauterine Growth Retardation
- Intracranial Hemorrhage
- Teratogen Exposure
- Perinatal infection (Chorioamnionitis)
- Multiple Gestation
- Postnatal causes or risk factors (<8 to 20%)
VI. Types
- Limb spasticity (80% of cases)
- Features
- Hypertonic Muscle movement
- Hyperreflexia
- Scissors gait
- Toe-walking
- Motor Impairment Categories
- Diplegia
- Hemiplegia
- Quadriplegia
- Features
-
Dyskinesia (10-20% of cases)
- Slow, writhing, uncontrollable limb movements worse during stress
- Ataxic Cerebral Palsy (5-10% of cases)
- Wide-based gait
- Intention Tremor
- Mixed Cerebral Palsy
- Combination or other features
VII. Associated Conditions
- Coordination and Movement Disorder
- Features vary depending on Cerebral Palsy type (see above)
- Typically limb spasticity, Dyskinesia or Ataxia
- Sensory deficits
- 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
- Screen for Strabismus and Hemianopia
- Gastrointestinal disorders
- Vomiting (Delayed Gastric Emptying)
- Constipation (GI motility, Dehydration, mobility)
- Risk of Sigmoid Volvulus
-
Swallowing difficulty
- Aspiration risk
- Drooling
- Inadequate oral intake
- Osteoporosis (90%)
- Hip displacement (33%)
- Urinary Incontinence (25%)
- Behavioral or emotional disorders
- Sleep Disorder
VIII. Diagnosis
- Systematic, stepwise approach to diagnosis
- Thorough history and physical
- Recognize permanent, non-progressive motor function disorder
- Evaluate for comorbidities associated with Cerebral Palsy
- Obtain imaging if perinatal Ultrasound is not sufficient for diagnosis (see below)
- Consider differential diagnosis (see below)
- Findings suggestive of Cerebral Palsy
- Slow motor development
- Altered Muscle tone and Posture
- Moro Reflex persists beyond 6 months of age
- Dominant hand preference established under 12 months
- Findings of alternative diagnosis (neurodegenerative)
- Loss of acquired skills
- Atypical body odor (as seen in metabolic disorders)
- Loss of Deep Tendon Reflexes
- Severity Assessment Tools
- Gross Motor Function Classification System (CMFCS) for Cerebral Palsy
- Age based tool that rates mobility, Posture, and balance each on 5 point scale (1 for mild, 5 for severe)
- https://cerebralpalsy.org.au/our-research/about-cerebral-palsy/what-is-cerebral-palsy/severity-of-cerebral-palsy/gross-motor-function-classification-system/
- Palisano (1997) Dev Med Child Neurol 39:214-23 [PubMed]
- Gross Motor Function Classification System (CMFCS) for Cerebral Palsy
- Timing of diagnosis
- Cerebral Palsy may be suspected on perinatal Ultrasound or postnatal Brain MRI <6 months
- Diagnosis may not be made until age 12-24 months in areas with fewer Imaging Resources
- Resources
IX. Imaging
- Perinatal Ultrasound (Fetal Survey)
- Newborn transcranial Ultrasound
-
Brain MRI (variable findings depending on cause)
- Schizocephaly (cerebral tissue clefts)
- Hydrocephalus
- Periventricular leukomalacia
X. Differential diagnosis
- Consider neurodegenerative disorders and Inborn Errors of Metabolism
- Examples
- Arginase deficiency
- Glutaric aciduria
- Niemann-Pick Disease
- Lesch-Nyhan Syndrome
- Rett Syndrome
XI. Management
- Approach
- Involve a multispecialty care team (surgery, OT, PT, speech-language, social work, psychology)
- Assistive Devices for Activities of Daily Living
- Enable mobility, optimize functionality and independence
- Address Mood Disorders
- Establish realistic goals with family
- Expect nearly best motor development by age 5 years
- Goals change over time (e.g. communication, social, academic)
- Global therapies with variable efficacy
- Neurodevelopmental treatment (Bobath method)
- Conductive education with rehabilitation program
- Communication
- Speech therapy may aid communication (e.g. voice synthesizers)
-
Hearing
- Screen Hearing every 6 months from age 6 months to 3 years
-
Vision
- Screen Visual Acuity at 1 and 4 years
- Observe for Strabismus and Hemianopia
- Physical therapy
- Balance benefit with stress of frequent visits
- Improve hand function
- Constraint induced movement therapy
- Dominant hand constrained to promote use of nondominant hands
- May be more frustrating technique for children
- Hand-arm intensive bimanual therapy
- Also encourages use of both hands
- Constraint induced movement therapy
- Resistive Exercise is controversial
- Has been avoided due to increased spasticity risk
- Recent studies suggest strengthening is beneficial
- Medications: Spasticity
- Botulinum Toxin (Botox) injections for leg spasticity
- Baclofen (Lioresal) intrathecal
- Medications: General
- Pain (especially Hip Pain)
- Assess pain and treat adequately
- Hip Dislocation may occur spontaneously due to spasticity
- Constipation
- Bowel regimen (fluids, fiber, Stool Softeners)
- Osteoporosis
- Consider DEXA Scan (adults) and Osteoporosis treatment to prevent Fractures
- Pain (especially Hip Pain)
-
Seizures
- Emergent Seizure management is the same as for non-Cerebral Palsy patients
- Administer Benzodiazepines and Anticonvulsant loading
- Check Serum Glucose and anticonvulsant levels
- Other labs and diagnostic evaluation only as indicated by exam and history
- Seizures may be refractory to standard measures
- Combination antiepileptics are often required for Seizure control
- Consider Ketogenic Diet (risk of Drug Interactions)
- Emergent Seizure management is the same as for non-Cerebral Palsy patients
- Surgery
- Leg spasticity
- Selective dorsal rhizotomy
- Selective cutting of L1-S2 dorsal rootlets
- Selective dorsal rhizotomy
- Muscle imbalance with hip subluxation, dislocation
- Abduction bracing
- Soft tissue release
- Femoral or pelvic osteotomy
- Implantable stimulator to superior-medial Cerebellum
- Gastrostomy for Swallowing and eating difficulties
- Leg spasticity
-
Urinary Incontinence
- Consider physical therapy, biofeedback and medications
- Consider Bladder stimulators (neuromodulation)
- Prevent Pressure Ulcers
- Optimize repositioning and support surfaces
- Early wound care Consultation for Pressure Sores
- Adjuncts
XII. Prevention: Secondary conditions
- Observe for cancers of higher risk in Cerebral Palsy
- Brain cancer
- Breast Cancer
- Routine Health Maintenance
- Left lateral position more comfortable for pelvic
- Educate about injury risks