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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
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Causes and clinical features of cerebral palsy subtypes

Causes and clinical features of cerebral palsy subtypes
  Proportion of CP cases Common causes Infants affected Common clinical features
Infants and young children Children >5 years old
Spastic subtypes        
  • Increased tone
  • Signs of upper motor neuron syndrome
    • Brisk deep-tendon reflexes
    • Extensor plantar response
    • Clonus
  • Contractures of affected muscles
Spastic diplegia* 13 to 25%
  • Most commonly associated with PVL
  • Preterm infants
  • Risk increases with decreasing gestational age
  • First few months – Hypotonia of the lower limbs with delayed functional maturation
  • By 6 months – Spasticity involving ankle plantar flexors and hip adductors
  • Crawling may be combat style
  • Lower limbs are more affected than the upper limbs
  • Affected patients have flexion, adduction, and internal rotation of the hips with contractures of the hip flexors and hamstring muscles
  • Variable degrees of flexion at the elbows and knees
  • Reduced limb length and muscle bulk in lower extremities
Spastic hemiplegia* 21 to 40%
  • Neonatal stroke
  • Prenatal circulatory disturbances
  • Brain maldevelopment
  • Term infants of normal birth weight
  • Motor asymmetry (may not be apparent in the newborn period)
  • Early (before age 12 months) hand dominance
  • Inability to use both hands in midline or to reach out with the affected limb
  • Abnormal posturing on one side
  • In prone position, the affected upper limb provides decreased support, and movement of the affected leg is diminished
  • In a sitting position, the affected leg tends to extend
  • Protective reactions that appear at 5 to 8 months of age are asymmetric
  • Over first 1 to 2 years, movement and tone on the affected side typically decrease before tone and tendon reflexes abnormally increase
  • Typical posture (see description to the right) appears by age 2 years in most cases
  • One side of the body is affected
  • The arm typically is more affected than the leg
  • The arm is adducted at the shoulder and flexed at the elbow, the forearm is pronated, and the wrist and fingers are flexed with the hand closed
  • The hip is partially flexed and adducted, and the knee and ankle are flexed; the foot may remain in the equinovarus or calcaneovalgus position
  • Most children also have sensory deficits
  • In mildly affected patients, postural abnormalities are more apparent during walking or running; however, unless severe intellectual disability is present, independent walking usually occurs at the appropriate age or is only slightly delayed
Spastic quadriplegia* 20 to 43%
  • Congenital infection
  • Cerebral dysgenesis
  • Perinatal or postnatal events
  • Most commonly term SGA infants, but can also occur in preterm infants
  • Moderate or severe psychomotor delay
  • Poor head control
  • Spasticity may begin by 2 to 3 months of age
  • Adduction of the thighs results in typical scissoring of the legs
  • By 9 to 10 months of age, infants when pulled to sitting are unable to flex the legs and have poor truncal balance
  • All limbs are affected
  • Upper limbs may be equally or more involved than lower limbs
  • Children often have severe functional impairment
  • Feeding difficulties, chronic respiratory insufficiency, and seizure disorder are common
Dyskinetic subtypes 12 to 14%
  • Most cases are caused by severe perinatal asphyxia resulting in injury to the thalamus, basal ganglia, hippocampus, reticular formation, and/or cerebellum
  • Severe hyperbilirubinemia (kernicterus) can cause choreoathetotic CP
  • Predominantly term infants
In early infancy:
  • Reduced spontaneous movement
  • Hypotonia at rest, variable tone with movement or emotion
  • Oromotor incoordination
  • Persistence of primitive reflexes
  • Involuntary grimacing
  • Drooling
  • Delayed psychomotor development
  • Head can be persistently turned

Age 2 to 3 years:

  • Involuntary movements are apparent
  • Abnormal posturing:
    • Extension patterns in the supine position
    • Flexion with shoulder retraction in the prone position
    • Head usually is persistently turned to one side
  • Involuntary movements
  • Contractures are not common but may evolve later in life
  • Variable degree of dysarthria and intellectual disability
Choreoathetotic CP:
  • Chorea consists of rapid, irregular, unpredictable contractions of individual muscles or small muscle groups that involve the face, bulbar muscles, proximal extremities, and fingers and toes
  • Athetosis consists of slow, smooth, writhing movements that involve distal muscles
  • Movements may be induced or accentuated by emotion or change in posture
  • Athetosis is most apparent during reaching
  • Stress, excitement, or fever may exacerbate chorea
  • Primitive reflexes often are retained
  • Oropharyngeal difficulties occur commonly
Dystonic CP:
  • Repetitive, patterned, twisting, and sustained movements of the trunk and limbs that may be either slow or rapid
  • Pyramidal signs and anarthria may occur
  • "Tension," a sudden involuntary increase in tone affecting both flexor and extensor muscles, may occur during attempted movement or with emotion
  • Tendon reflexes are normal or may be difficult to elicit
  • Clonus and extensor plantar responses are absent
Ataxic CP 4 to 13%
  • Most cases are caused by early prenatal events
  • Etiology is frequently unknown
  • Some cases have genetic causes, including:
    • Cerebellar hypoplasia
    • Granule cell deficiency
    • Joubert syndrome
  • Rarely associated with congenital hypoplasia of the cerebellum
  • Term infants
  • Hypotonia and incoordination
  • Motor milestones and language skills typically are delayed
  • Ataxic movements
  • Widespread disorder of motor function
  • Ataxia usually improves with time
  • Speech typically is slow, jerky, and explosive

CP: cerebral palsy; PVL: periventricular leukomalacia; SGA: small for gestational age.

* The suffix "paresis" denotes weakness and "plegia" means paralysis. However, these terms often are used interchangeably and do not necessarily imply a difference in severity.

¶ A separate category of "hypotonic CP" (also called "atonic CP") has been described, though it is generally absent from contemporary classifications. The majority of patients with "hypotonic CP" in early infancy later develop spastic, dyskinetic, and particularly ataxic CP.
References:
  1. Surveillance of Cerebral Palsy in Europe. Surveillance of cerebral palsy in Europe: a collaboration of cerebral palsy surveys and registers. Surveillance of Cerebral Palsy in Europe (SCPE). Dev Med Child Neurol 2000; 42:816.
  2. Noritz GH, Murphy NA, Neuromotor Screening Expert Panel. Motor delays: early identification and evaluation. Pediatrics 2013; 131:e2016.
  3. Odding E, Roebroeck ME, Stam HJ. The epidemiology of cerebral palsy: incidence, impairments and risk factors. Disabil Rehabil 2006; 28:183.
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