Cerebral Palsy
Cerebral Palsy: Excerpt from The 5-Minute Pediatric Consult
Stephen Contompasis, MD
Cerebral Palsy - BASICS
Cerebral Palsy - description
Cerebral palsy (CP) describes a group of disorders of movement and posture, limiting activity, attributed to nonprogressive underlying brain pathology. The motor disorders of CP are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior, or by a seizure disorder.
- Spastic (pyramidal; 40%): Increased deep tendon reflexes, sustained clonus, hypertonia, and the clasp-knife response:
- Spastic diplegia–lower extremity involvement
- Spastic hemiplegia–1 side of the body involved
- Spastic quadriplegia–total body involvement
- Dyskinetic (30%): Fluctuating tone, rigid total body involvement by definition. Persistent primitive reflex patterns (asymmetric tonic neck reflex, labyrinthine)
- Athetoid: Slow writhing movements (or chorea; rapid, random, jerky movements)
- Dystonic: Posturing of the head, trunk, and extremities
- Ataxic (<10%): Characterized by cerebellar signs (ataxia, dysmetria, past pointing, tremor, nystagmus) and abnormalities of voluntary movement
- Mixed (10%): 2 or more types codominant, most often spastic and dyskinetic
- Other (10%): Criteria for CP met, but specific subtype cannot be defined
- Extrapyramidal: Sometimes applied to nonspastic types of CP as a group
Cerebral Palsy - epidemiology
- ~50% of cases are associated with prematurity.
- Increased concordance among monozygotic versus dizygotic twins in some studies (not in others)
- Intrauterine growth retardation (IUGR) more common in CP than controls, especially for full-term infants in whom CP develops
- Male > Female (1.3:1)
- Inconsistent relationship to maternal age, socioeconomic status, and parity
- Prenatal factors are more strongly associated with subsequent CP than are perinatal or postnatal factors; however, individual risk factors are poorly predictive of subsequent CP in the individual child.
- Diagnosis of perinatal asphyxia requires evidence of hypoxic–ischemic insult, severe encephalopathy (e.g., neonatal seizures, severe hypotonia), consistent laboratory/radiological findings; accounts for only ~9% of cases of CP.
Cerebral Palsy - incidence prevalence
- Increased with multiple gestation (10% were twins in 1 study)
- Prevalence ~2/1000
Cerebral Palsy - etiology
- Not apparent in most cases. A more recently recognized perinatal factor is the presence of chorioamnionitis; mild or even subclinical cases may have increased association with CP.
- Epidemiologic studies indicate 2 types of vulnerability to CP:
- Prematurity: Vulnerability of the periventricular white matter between 28 and 32 weeks of gestation results in periventricular leukomalacia.
- IUGR: Fetal growth retardation associated with CNS dysgenesis, non-CNS malformation, teratogens, growth retardation, evidence of hypoxic-ischemic encephalopathy more often seen
Cerebral Palsy - associated conditions
- Sensory:
- Sensorineural and conductive hearing loss
- Impaired visual acuity
- Oculomotor dysfunction
- Strabismus
- Cortical visual impairment
- Somatosensory impairments
- Cognitive and linguistic:
- Mental retardation (MR) in ~50%, especially in spastic quadriparesis
- High incidence of language and learning disabilities
- Dysarthria
- Attention deficit hyperactivity disorder
- Sleep and behavioral disturbances
- Neurologic:
- Musculoskeletal:
- Contractures
- Hip subluxation/dislocation
- Scoliosis
- Cardiorespiratory:
- Upper airway obstruction
- Aspiration pneumonitis
- Restrictive lung disease/thoracic deformity
- Reactive airway disease
- GI/nutritional:
- Poor growth
- Gastroesophageal reflux
- Constipation
- Oral motor dysfunction/dysphagia
- Urinary: Neurogenic bladder
- Skin: Decubitus ulcers
- Dental:
- Malocclusions
- Caries
- Gingival hyperplasia
- Abnormalities of enamel (congenital)
Cerebral Palsy - DIAGNOSIS
Cerebral Palsy - signs & symptoms
Cerebral Palsy - history
- Prenatal:
- Exposure to toxins/drugs
- Infections or fever
- HIV/STD risk
- Vaginal bleeding
- Abnormal fetal movement
- Pre-eclampsia (especially proteinuria)
- Breech position
- Poor maternal weight gain
- Premature labor
- Fetal distress
- IUGR
- Prenatal testing
- Placental disorders
- Perinatal:
- Premature delivery
- Neonatal resuscitation
- Low Apgar scores (<5 at 5 minutes)
- Birth trauma
- Evidence of neonatal encephalopathy (seizures, severe hypotonia)
- Complicated neonatal course (intraventricular hemorrhage, prolonged respiratory support, meningitis, sepsis, hyperbilirubinemia)
- Postnatal:
- Hospitalization for severe infection or trauma
- Periodic or persistent deterioration in function (suggests neurodegenerative/metabolic disease)
- Development:
- Severe delay in motor milestones (e.g., not rolling at 7 months, not sitting at 8 months, not walking at 15 months) associated with persistent primitive reflexes (e.g., prominent tonic neck and labyrinthine responses at 1 year of age) and delayed or absent development of protective reactions (e.g., lateral prop at 7 months, parachute at 13 months)
- Associated delays in language, play, social, and adaptive behavior
Cerebral Palsy - physical exam
- Respiratory pattern: Obstruction, aspiration risk, evidence of dysmorphism/pigmentary skin changes, and growth abnormalities contribute to assessment of etiology.
- Head circumference: To evaluate for microcephaly/macrocephaly/hydrocephaly; growth velocity is important.
- Strabismus/cataracts/iris or retinal abnormalities: Eye exam: Cranial nerve damage, muscle imbalance, metabolic disease, or congenital infection
- Musculoskeletal:
- Decreased range with contractures
- Leg-length discrepancy: Hip dislocation
- Spinal curvature/scoliosis
- Neurologic:
- Documentation of best level of visual motor/manipulative skills (e.g., able to run, transfer, hold a cup): To follow course of motor impairment
- Cranial nerves: Strabismus, speech and swallowing, vision and hearing
- Tone: Spasticity versus rigidity versus hypotonia
- Strength: Often decreased
- Hyperactive deep tendon reflexes and clonus in spasticity; Babinski reflex (extensor response to plantar stimulation)
- Persistent primitive reflexes
- Protective reactions: Head and trunk righting, prop reactions, parachute; cerebellar signs
- Balance, stability
Pitfalls:
- Overdiagnosis of CP in infants with spastic hypertonia; normalization of tone/function may take up to 2 years.
- False or premature assumption of cognitive deficit in children with severe dysarthria. May take years of augmentative communication supports to determine true potential.
- Slowly progressive neurogenerative disease may masquerade as CP.
- Cervical cord lesions may masquerade as quadriparetic spastic CP.
- Determination of ideal body weight may be complex in CP; growth standards according to CP type are under development.
Cerebral Palsy - tests
- Hearing and vision: All in 1st year, with regular follow-up exams
- Audiologic evaluation: Required for children with CP in accordance with regular health care and educational guidelines
- Urodynamic studies: Spastic bladder in those with recurrent UTIs or voiding dysfunction
- Sleep study: May disclose treatable obstructive sleep apnea in those with somnolence or abnormal sleep-wake cycles
- Pulmonary function studies: Document progressive restrictive pulmonary dysfunction (e.g., in severe scoliosis)
- Brain wave (EEG): If seizure suspected
Cerebral Palsy - lab
- Genetic and metabolic studies: If history or physical suggests a progressive or hereditary disorder
- Blood chemistries, liver function studies, cell counts: Evaluate nutritional/metabolic status, anticonvulsant levels
Cerebral Palsy - imaging
- Brain imaging: Perform when hydrocephalus is suspected; can help determine etiology
- Radiography: Should be done routinely in spastic diparesis for hip dislocation; consider scoliosis films
- Radionuclide studies to evaluate gastroesophageal reflux, gastric emptying, aspiration
Cerebral Palsy - differencial diagnosis
- Motor syndromes related to spinal cord, lower motor neuron, peripheral nerve, primary muscular disease, or progressive disorders of the basal ganglia (dopa-responsive dystonia)
- Connective tissue disorders (primary and secondary) resulting in musculoskeletal abnormalities (e.g., arthrogryposis multiplex, skeletal dysplasias)
- Inborn errors of metabolism and CP: Protean manifestations, dyskinesia, ataxia, postnatal growth failure, neurologic deterioration, recurrent vomiting
Cerebral Palsy - TREATMENT
Cerebral Palsy - general measures
- Family-centered care is directed toward optimizing function and minimizing handicap.
- Interdisciplinary clinics: Services (medical, surgical, therapy) coordinated with primary physician
- Therapy with IM injections of botulinum toxin used increasingly, although the therapy may need to be repeated every 3–5 months. Establish specific goals for therapy.
- Education services: Recent emphasis on inclusion/mainstreaming; for many, special education services are still required.
- Augmentative communication supports especially for nonverbal/dysarthric children
- Physical, occupational, speech/language therapy, other allied health professionals: Therapy provided in home, school, and hospital settings; directed primarily at improved mobility, self-care, and communication; orthodontists for braces
- Counseling support for children coping with chronic disability
- Social services: Provided in a variety of contexts to aid in the coordination of care
- Vocational counseling and employment options, assistance with transition to adulthood, self advocacy, self determination
- Transition to adult health care system
Cerebral Palsy - diet
Nutritional assessment and support for those with dysphagia or poor growth
Cerebral Palsy - FOLLOW UP
- Requirements for follow-up vary greatly with the degree of disability and impairment. An interdisciplinary clinic setting may be more appropriate for a child with severe CP.
- Early referral to a pediatric orthopedist is indicated, especially for monitoring of early hip subluxation, which is best managed before progression to dislocation.
- Early referral for developmental assessment: Need for early intervention, to optimize development and promote family coping.
Cerebral Palsy - bibliography
- 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;62:851–863.
- Bax M, Goldstein M. et al. Proposed definition and classification of cerebral palsy. Dev Med Child Neurol. 2005;47:571–576.
- Petersen MC, Palmer FB. Advances in prevention and treatment of cerebral palsy. Ment Retard Dev Disabil Res Rev. 2001;7:30–37.
- Wu YW, Colford JM Jr. Chorioamnionitis as a risk factor for cerebral palsy: A meta-analysis. JAMA. 2000;284:1417–1424.
Cerebral Palsy - CODES
Cerebral Palsy - icd9
343.9 Infantile cerebral palsy, unspecified
Cerebral Palsy - PATIENT TEACHING-MED
Online information for parents: United Cerebral Palsy Association, www.ucp.org.
Cerebral Palsy - FAQ
- Q: Is severe clumsiness a form of CP?
- A: Mild spastic diplegia or hemiplegia may present this way, but tone abnormalities distinguish these from developmental coordination disorders.
- Q: Do children with CP also have MR?
- A: Only ~50% have MR.
- Q: What about surgery for CP?
- A: Spasticity in the lower extremities may be addressed directly with selective dorsal rhizotomy. Otherwise, surgical therapy in CP is directed at associated conditions (strabismus, gastric tube placement).
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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