> Table of Contents > Cerebral Palsy
Cerebral Palsy
Christina Mezzone, DO, MS
Maria Lombardi, DO
image BASICS
DESCRIPTION
Cerebral palsy (CP) is a group of clinical syndromes characterized by motor and postural dysfunction due to permanent and nonprogressive disruptions in the developing brain. Motor impairment resulting in activity limitation is necessary for this diagnosis. CP is classified by the nature of the movement disorder and its functional severity. Individuals with this disorder are affected with secondary musculoskeletal and neurologic problems (intellectual, sensory, speech and language impairment, and seizures).
EPIDEMIOLOGY
Incidence
  • Overall, 1.5 to 2.5/1,000 live births
  • Incidence increases as gestational age (GA) at birth decreases:
    • 146/1,000 for GA of 22 to 27 weeks
    • 62/1,000 for GA of 28 to 31 weeks
    • 7/1,000 for GA of 32 to 36 weeks
    • 1/1,000 for GA of 37+ weeks
Prevalence
3 to 4/1,000 of the population
ETIOLOGY AND PATHOPHYSIOLOGY
  • Multifactorial; CP results from static injury or lesions in the developing brain, occurring prenatally, perinatally, or postnatally.
  • Neuropathology linked to GA at time of brain insult
  • Cytokines, free radicals, and inflammatory response are likely contributing factors.
  • Etiology is most likely multifactorial and depends on timing of brain insult: prenatally, perinatally, or postnatally (see “Risk Factors”).
  • Spastic CP is most common, usually related to premature birth, with either periventricular leukomalacia or germinal matrix hemorrhage.
  • Dystonic or athetotic CP, often resulting from kernicterus, is now rare due to improved management of hyperbilirubinemia.
Genetics
There are reports of associations between CP and polymorphisms of certain genes: thrombophilic, cytokines, and apolipoprotein E.
RISK FACTORS
  • Prenatal: congenital anomalies, multiple gestation, in utero stroke, intrauterine infection (cytomegalovirus [CMV], varicella), intrauterine growth retardation (IUGR), clinical and histologic chorioamnionitis, antepartum bleeding, maternal factors (cognitive impairment, seizure disorders, hyperthyroidism), abnormal fetal position (e.g., breech)
  • Perinatal: preterm birth, low birth weight, periventricular leukomalacia, perinatal hypoxia/asphyxia, intracranial hemorrhage/intraventricular hemorrhage, neonatal seizure or stroke, hyperbilirubinemia
  • Postnatal: traumatic brain injury or stroke, sepsis, meningitis, encephalitis, asphyxia, and progressive hydrocephalus
GENERAL PREVENTION
  • Treating mothers with magnesium sulfate during preterm delivery is neuroprotective for fetus and may reduce the risk of CP. Effect on term fetus is unknown.
  • Improved management of hyperbilirubinemia with decrease in kernicterus has greatly reduced dyskinetic CP.
  • Prevention or reduction of chorioamnionitis and premature births
COMMONLY ASSOCIATED CONDITIONS
  • Seizure disorder (22-40%)
  • Intellectual impairment (23-44%)
  • Behavioral problems
  • Speech and language impairment (42-81%)
    • May have an impact on expressive and/or receptive language
    • May be nonverbal
  • Sensory impairments
    • Hearing deficits
    • Visual (62-71%): poor visual acuity, strabismus (50%), or hemianopsia
  • Feeding impairment, swallowing dysfunction, and aspiration: when severe, may require gastrostomy feedings
  • Poor dentition, excessive drooling
  • GI conditions: constipation (59%), vomiting (22%), gastroesophageal reflux
  • Decreased linear growth and weight abnormalities (under- and overweight)
  • Osteopenia
  • Bowel and bladder incontinence
  • Orthopedic: contractures, hip subluxation/dislocation, scoliosis (60%)
image DIAGNOSIS
  • A clinical diagnosis including
    • Delayed motor milestones
    • Abnormal tone
    • Abnormal neurologic exam suggesting a cerebral etiology for motor dysfunction
    • Absence of regression (not losing function)
    • Absence of underlying syndromes or alternative explanation for etiology
  • Although the pathologic lesion is static, clinical presentation may change as the infant grows and develops.
  • Accurate early diagnosis remains difficult. Neurologic abnormalities observed in the first 1 to 2 years of life may resolve; be cautious of diagnosing CP before age 2 years.
  • Serial exams are often required for a definitive diagnosis.
PHYSICAL EXAM
  • Assess for more than one type of neurologic impairment:
    • Spasticity: increased tone/reflexes/clonus
    • Dyskinesia: abnormal movements
    • Hypotonia: decreased tone
    • Ataxia: abnormal balance/coordination
  • Areas of exam
    • Tone: may be increased or decreased
    • Trunk and head control: often poor but may be advanced due to high tone
    • Reduced strength and motor control
    • Persistence of primitive reflexes
    • Asymmetry of movement or reflexes
    • Decreased joint range of motion and contractures
    • Brisk deep tendon reflexes
    • Clonus
    • Delayed motor milestones: serial exams most effective
    • Gait abnormalities: scissoring, toe-walking
  • CP is classified by the following:
    • Muscle tone or movement disorder
      • Spasticity
        • Unilateral: hemiplegic
        • Bilateral: diplegic (lower extremity [LE] > upper extremity [UE] involvement) or quadriplegic (UE ≥ LE involvement)
      • Dystonia: hypertonia and reduced movement
      • Choreoathetosis: irregular spasmodic involuntary movements of the limbs or facial muscles
      • Ataxia: loss of orderly muscular coordination
    • Motor function severity
      • The Gross Motor Function Classification System (GMFCS) scores I-IV
        • Score of I: ambulates without limitation
        • Score of II: ambulates without assistive devices but some limitation
        • Score of III: ambulates with assistive mobility devices
        • Score of IV: Self-mobility is limited, but technology can help.
        • Score of V: Self-mobility is severely limited, even with technology.
      • The Manual Ability Classification System (MACS) can be used to assess upper extremity and fine motor function.
DIFFERENTIAL DIAGNOSIS
Benign congenital hypotonia, brachial plexus injury, familial spastic paraplegia, dopa-responsive dystonia, transient toe-walking, muscular dystrophy, metabolic disorders (e.g., glutaric aciduria type 1), mitochondrial disorders, genetic disorders (e.g., Rett syndrome)
DIAGNOSTIC TESTS & INTERPRETATION
CP is a clinical diagnosis based on history, physical, and risk factors. Laboratory testing is not needed to make diagnosis but can help exclude other etiologies.
  • Testing for metabolic and genetic syndromes (1)[C]
    • Not routinely obtained in the evaluation for CP.
    • Considered if no specific etiology is identified by neuroimaging or there are atypical features in clinical presentation.
    • Detection of certain brain malformations may warrant genetic or metabolic testing to identify syndromes.
  • Screening for coagulopathies: Diagnostic testing for coagulopathies should be considered in children with hemiplegic CP with cerebral infarction identified on neuroimaging (1)[C].
Initial Tests (lab, imaging)
  • Neuroimaging is not essential, but it is recommended in children with CP for whom the etiology has not been established (1)[C].
  • MRI is preferred to CT if need to determine etiology and timing of a brain insult (1)[C].
  • P.177

  • Abnormalities found in 80-90% of patients: brain malformation, cerebral infarction, intraventricular or other intracranial hemorrhage, periventricular leukomalacia, ventricular enlargement, or other CSF space abnormalities
Diagnostic Procedures/Other
  • The Communication Function Classification System has recently been developed as another means of assessing verbal performance.
  • International Classification of Functioning, Disability and Health for CP have been newly developed to standardize functional assessments.
  • Screening for comorbid conditions: developmental delay/intellectual impairment, vision/hearing impairments, speech and language disorders, feeding/swallowing dysfunction, or seizures
  • EEGs should only be obtained if there is a history of suspected seizures.
Test Interpretation
Perinatal brain injury may include the following:
  • White matter damage
    • Most common in premature infants
    • Periventricular leukomalacia: gliosis with or without focal necrosis with resulting cysts and scarring; may be multiple lesions of various ages. Necrosis can lead to cysts/scarring.
    • Germinal matrix hemorrhage: may lead to intraventricular hemorrhage
  • Gray matter damage: more common in term infants; cortical infarcts, focal neuronal damage, myelination abnormalities
image TREATMENT
Focuses on control of symptoms; treatments reduce spasticity to prevent painful contractures, manage comorbid conditions, and optimize functionality and quality of life.
GENERAL MEASURES
  • Early intervention programs for preterm infants influences motor and cognitive outcomes (2)[A].
  • Referral to early intervention for children ages 0 to 3 years is essential.
  • Various therapy modalities enhance functioning:
    • Physical therapy to improve posture stability and gait, motor strength and control, and prevent contractures
    • Occupational therapy to increase functional activities of daily living and other fine motor skills
    • Speech therapy for verbal and nonverbal speech and to aid in feeding
  • Equipment optimizes participation in activities:
    • Orthotic splinting (ankle-foot orthosis) maintains functional positioning and prevents contractures.
    • Spinal bracing (body jacket) may slow down scoliosis.
    • Augmentative communication with pictures, switches, or computer systems for nonverbal individuals
    • Therapeutic and functional electrical stimulation decreases activity limitation in gait.
    • Use of adaptive equipment such as crutches, walkers, gait trainers, and wheelchairs for mobility and standers for weight bearing
MEDICATION
First Line
  • Diazepam (3)[A]
    • Short-term treatment for generalized spasticity; insufficient evidence on motor function
    • A γ-aminobutyric acid-A (GABAA) agonist that facilitates CNS inhibition at spinal and supraspinal levels to reduce spasticity
    • Adverse effects: ataxia and drowsiness
    • Adult dose: 2 to 12 mg/dose PO q6-12h
    • Pediatric dose (<12 years and <15 kg): <8.5 kg: 0.5 to 1 mg HS; 8.5 to 15 kg: 1 to 2 mg HS; children 5 to 16 years of age and ≥15 kg: 1.25 mg TID
  • Botulinum toxin type A (3)[A]
    • Acts at neuromuscular junction to inhibit the release of acetylcholine to reduce tone
    • Injected directly into muscles of interest for localized spasticity; insufficient evidence on motor function
    • Higher functional benefit when combined with occupational therapy
    • Lasts for 12 to 16 weeks following injection
Second Line
  • Baclofen (3)[A]
    • GABAB agonist, facilitates presynaptic inhibition of mono- and polysynaptic reflexes
    • Adverse effects: drowsiness and sedation
    • Abrupt withdrawal symptoms: spasticity, hallucinations, seizures, confusion, hyperthermia
    • Adults: Initial dose is 5 mg TID; increase dosage every 3 days to an average maintenance dose of 20 mg TID 80 mg/day maximum
    • Pediatric dose (>2 years): initial 10 to 15 mg/day. Titrate to effective dose (maximum 40 mg/day). <8 years old: 60 mg/day maximum; >8 years old: 60 mg/day maximum
  • Intrathecal baclofen (baclofen pump) (4)[A]
    • Continuous intrathecal route allows greater maximal response with smaller dosage to reduce spasticity.
    • May help ambulatory individuals with gait, but no improvement is seen in nonambulatory patients.
    • Adverse effects: infection, catheter malfunction, CSF leakage
ADDITIONAL THERAPIES
  • Multidisciplinary care including ophthalmology; neurology; orthopedics; physiatry along with physical, occupational, and speech therapists
  • A primary care “medical home” that coordinates medical and community services, provides support for the patient and the patient's family
SURGERY/OTHER PROCEDURES
  • Dorsal root rhizotomy selectively cuts dorsal rootlets from L1-S2. Best for patients with normal intelligence with spastic diplegia. Decreases spasticity in lower limbs when done in conjunction with physiotherapy but associated with adverse effects. Evidence is lacking as to long-term outcomes.
  • Surgical treatment of joint dislocations/subluxation, scoliosis management, tendon lengthening, gastrostomy
COMPLEMENTARY & ALTERNATIVE MEDICINE
  • Hyperbaric oxygen is controversial and not recommended in those who do not suffer hypoxic ischemic encephalopathy (5)[A].
  • Therapeutic horse riding or hippotherapy improves postural control and balance.
  • Aquatherapy improves gross motor function in patients with various motor severities (6)[B].
image ONGOING CARE
PROGNOSIS
Reduced lifespan strongly associated with level of functional impairment and intellectual disability
REFERENCES
1. Ashwal S, Russman BS, Blasco PA, et al. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2004;62(6):851-863.
2. Spittle A, Orton J, Anderson P, et al. Early developmental intervention programmes post-hospital discharge to prevent motor and cognitive impairments in preterm infants. Cochrane Database Syst Rev. 2012;(12):CD005495.
3. Delgado MR, Hirtz D, Aisen M, et al. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2010;74(4):336-343.
4. Pin TW, McCartney L, Lewis J, et al. Use of intrathecal baclofen therapy in ambulant children and adolescents with spasticity and dystonia of cerebral origin: a systematic review. Dev Med Child Neurol. 2011;53(10):885-895.
5. Lacey DJ, Stolfi A, Pilati LE. Effects of hyperbaric oxygen on motor function in children with cerebral palsy. Ann Neurol. 2012;72(5):695-703.
6. Lai CJ, Liu WY, Yang TF, et al. Pediatric aquatic therapy on motor function and enjoyment in children diagnosed with cerebral palsy of various motor severities. J Child Neurol. 2015;30(2):200-208.
Additional Reading
  • Himpens E, Van den Broeck C, Oostra A, et al. Prevalence, type, distribution, and severity of cerebral palsy in relation to gestational age: a meta-analytic review. Dev Med Child Neurol. 2008;50(5):334-340.
  • Nguyen TM, Crowther CA, Wilkinson D, et al. Magnesium sulphate for women at term for neuroprotection of the fetus. Cochrane Database Syst Rev. 2013;(2):CD009395.
  • O'Shea TM. Diagnosis, treatment, and prevention of cerebral palsy. Clin Obstet Gynecol. 2008;51(4):816-828.
Codes
ICD10
  • G80.9 Cerebral palsy, unspecified
  • G80.1 Spastic diplegic cerebral palsy
  • G80.2 Spastic hemiplegic cerebral palsy
Clinical Pearls
  • Management should focus on maximizing functioning and quality of life using multidisciplinary team approach.
  • Regression of motor skills does not occur with CP.