Pediatrics Book

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Cerebral Palsy

Aka: Cerebral Palsy
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  1. Epidemiology
    1. Incidence: Up to 2.5 per 1000 U.S. born children
  2. Pathophysiology
    1. Brain injury occurring before age 3 to 5 years
    2. Results in non-progressive disorder of movement and Posture
    3. Wide variability in disease involvement and in degree of intellectual capacity
  3. Causes
    1. Idiopathic in 25% of cases
    2. Prenatal causes or risk factors: Up to 80% 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
    3. Postnatal causes (<20%)
      1. CNS Infection
      2. Head Trauma
      3. Hyperbilirubinemia
  4. Signs
    1. Limb spasticity (80% of cases)
      1. Hypertonic muscle movement
      2. Hyperreflexia
      3. Scissors gait
      4. Toe-walking
    2. Diskinesia (10-20% of cases)
      1. Slow, writhing limb movements worse during stress
    3. Ataxic Cerebral Palsy (5-10% of cases)
      1. Wide-based gait
      2. Intention Tremor
  5. Associated findings
    1. Cognitive Impairment (66% of cases)
    2. Motor Impairment
      1. Diplegia
      2. Hemiplegia
      3. Quadriplegia
    3. Seizure disorder (50-66% of cases, often spastic type)
    4. Growth delay
    5. Hearing Impairment
    6. Vision Impairment
      1. Screen for Strabismus and hemianopia
    7. Gastrointestinal disorders
      1. Vomiting (delayed gastric emptying)
      2. Constipation (GI motility, dehydration, mobility)
      3. Risk of sigmoid Volvulus
    8. Swallowing difficulty
      1. Aspiration risk
      2. Drooling
      3. Inadequate oral intake
    9. Osteoporosis
    10. Urinary Incontinence
    11. Altered sensory perception (pain on light touch)
  6. Diagnosis
    1. Assessment Tools
      1. Gross Motor Function Classification System for CP
        1. Palisano (1997) Dev Med Child Neurol 39:214-23
    2. 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
    3. 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
  7. Differential diagnosis
    1. Consider neurodegenerative disorders
    2. Examples
      1. Arginase deficiency
      2. Glutaric aciduria
      3. Niemann-Pick Disease
      4. Lesch-Nyhan Syndrome
      5. Rett Syndrome
  8. Management
    1. Global therapies with variable efficacy
      1. Neurodevelopmental treatment (Bobath method)
      2. Conductive education with rehabilitation program
    2. Physical therapy
      1. Balance benefit with stress of frequent visits
      2. 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
    3. Medications: Spasticity
      1. Botulinum toxin (Botox) injections for leg spasticity
        1. Reddigough (2002) Dev Med Child Neurol 44:820-7
      2. Baclofen (Lioresal) intrathecally
        1. Campbell (2002) Dev Med Child Neurol 44:660-5
    4. Medications: Pain (especially Hip Pain)
      1. Assess pain and treat adequately
    5. 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)
    6. 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
      4. Gastrostomy for swallowing and eating difficulties
        1. Samson-Fang (2003) Dev Med Child Neurol 45:415-26
    7. Adjuncts
      1. Lower limb orthoses (variable evidence)
      2. Elastic body suits (difficult compliance)
    8. General measures
      1. Multidisciplinary approach (care team)
      2. Assistive Devices for Activities of Daily Living
      3. Enable mobility
      4. Address Mood Disorders
  9. Prevention: Secondary conditions
    1. 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
    2. Educate about injury risks
      1. Drowning risk
      2. Motor vehicle accidents
  10. References
    1. Majoewsky (2012) EM:Rap 2(9): 4
    2. Krigger (2006) Am Fam Physician 73:91-102

Cerebral Palsy (C0007789)

Definition (NCI) A group of disorders affecting the development of movement and posture, often accompanied by disturbances of sensation, perception, cognition, and behavior. It results from damage to the fetal or infant brain.
Definition (CHV) birth injury of the brain nerve that controls body movement
Definition (CHV) birth injury of the brain nerve that controls body movement
Definition (CHV) birth injury of the brain nerve that controls body movement
Definition (MEDLINEPLUS)

Cerebral palsy is a group of disorders that affect a person's ability to move and to maintain balance and posture. The disorders appear in the first few years of life. Usually they do not get worse over time. People with cerebral palsy may have difficulty walking. They may also have trouble with tasks such as writing or using scissors. Some have other medical conditions, including seizure disorders or mental impairment.

Cerebral palsy happens when the areas of the brain that control movement and posture do not develop correctly or get damaged. Early signs of cerebral palsy usually appear before 3 years of age. Babies with cerebral palsy are often slow to roll over, sit, crawl, smile or walk. Some babies are born with cerebral palsy; others get it after they are born.

There is no cure for cerebral palsy, but treatment can improve the lives of those who have it. Treatment includes medicines, braces, and physical, occupational and speech therapy.

NIH: National Institute of Neurological Disorders and Stroke
Definition (MSH) A heterogeneous group of nonprogressive motor disorders caused by chronic brain injuries that originate in the prenatal period, perinatal period, or first few years of life. The four major subtypes are spastic, athetoid, ataxic, and mixed cerebral palsy, with spastic forms being the most common. The motor disorder may range from difficulties with fine motor control to severe spasticity (see MUSCLE SPASTICITY) in all limbs. Spastic diplegia (Little disease) is the most common subtype, and is characterized by spasticity that is more prominent in the legs than in the arms. Pathologically, this condition may be associated with LEUKOMALACIA, PERIVENTRICULAR. (From Dev Med Child Neurol 1998 Aug;40(8):520-7)
Definition (CSP) heterogeneous group of nonprogressive motor disorders caused by chronic brain injuries that originate in the prenatal period, perinatal period, or first few years of life; the four major subtypes are spastic, athetoid, ataxic, and mixed cerebral palsy, with spastic forms being the most common; the motor disorder may range from difficulties with fine motor control to severe spasticity in all limbs; spastic diplegia (Little disease) is the most common subtype, and is characterized by spasticity that is more prominent in the legs than in the arms.
Concepts Disease or Syndrome (T047)
MSH D002547
ICD10 G80, G80.9
SnomedCT 270488006, 128188000, 155024003, 267696006, 1178005
English Cerebral paralysis, Cerebral Palsy, PALSY, CEREBRAL, Cerebral palsied, CEREBRAL PALSY, PALSY CEREBRAL, Cerebral palsy, NOS, CP, Palsy cerebral, CP (Cerebral Palsy), Cerebral palsy NOS, Cerebral Palsy [Disease/Finding], Cerebral palsy, unspecified, Palsy;cerebral, cerebral palsy unspecified, cerebral paralysis, Cerebral palsy, CP - Cerebral palsy, Cerebral palsy (disorder), cerebral; paralysis, paralysis; cerebral, Cerebral palsy [Ambiguous], cerebral palsy
German Zerebralparese, zerebrale Kinderlaehmung, Infantile Zerebralparese, Kinderlähmung, zerebrale
Swedish Cerebral pares
Japanese ﾉｳｾｲﾏﾋ, 脳性小児麻痺, 脳性まひ, リトル病, 小児麻痺-脳性, 脳性マヒ, リットル病, 脳性麻痺, Little病
Czech mozek - obrna, infantilní cerebrální paréza, dětská mozková obrna, Mozková obrna
Spanish Cerebral palsy [Ambiguous], Cerebral palsy, parálisis cerebral (trastorno), parálisis cerebral, Parálisis cerebral, Parálisis Cerebral, Paralisis Cerebral
Finnish CP-oireyhtymä
Italian PC (Paralisi cerebrale), Paralisi cerebrale
Russian LITTLA BOLEZN', PARALICH TSEREBRAL'NYI, TSEREBRAL'NYI PARALICH, ЛИТТЛА БОЛЕЗНЬ, ПАРАЛИЧ ЦЕРЕБРАЛЬНЫЙ, ЦЕРЕБРАЛЬНЫЙ ПАРАЛИЧ
Croatian CEREBRALNA PARALIZA
French Paralysie cérébrale, Infirmité motrice cérébrale, Infirmité motrice d'origine cérébrale, IMC (Infirmité Motrice Cérébrale), IMOC (Infirmité Motrice d'Origine Cérébrale)
Polish Choroba Little'a, Dziecięce porażenie mózgowe, Porażenie mózgowe dziecięce
Hungarian Agyi paresis, Cerebralis paresis
Dutch cerebraal; verlamming, verlamming; cerebraal, hersenverlamming, Encefalopathie, infantiele, Infantiele encefalopathie, Little, ziekte van
Portuguese Paralisia cerebral, Paralisia Cerebral
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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