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By convention, the cerebral palsies
(CP) are nonprogressive disorders of tone, strength, movement, and posture
due to central nervous system (CNS) injury, the consequences of
which become apparent in infancy. The CNS lesions variously involve
pyramidal (cortical, subcortical, brainstem, spinal cord), extrapyramidal,
or cerebellar motor systems. The static nature of the CNS lesion
and public misunderstanding of the nature of CP has prompted the
coining of the not entirely synonymous alternative designation static
encephalopathy (SE). A cerebral palsy (CP) is a static
encephalopathy (SE) in which motor dysfunction must be present,
although individuals may also manifest disorders of intellect, attention,
memory, sensory, autonomic, and other neurologic modalities. They
may also be at risk for epilepsy and for dysfunction of respiratory,
gastrointestinal, and other nonneural systems.1-6
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Cerebral palsies are a subgroup of SE, a category that also includes
individuals whose static CNS injury may involve various patterns
of intellectual dysfunction unaccompanied by central motor system
deficits. Where only one or a few specific relatively mild cognitive
modalities are impaired, the qualifier learning disabled may
be applied, whereas more severe conditions affecting many or all
cognitive domains suggests the qualifier intellectually
disabled rather than the older term mentally retarded.
Limitations of variable degree in the social-pragmatic functions
of daily life may or may not be found in individuals with CP.
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The combination and degree of deficits in individuals with SE
or the large subgroup comprising CP is dependant on the location
and extent of underlying CNS injury. The clinical pattern is often
predictive of the site of lesions and may be predictive of underlying cause.
However, the deficits of most individuals with CP or SE remain of
uncertain pathogenesis, although rapid progress in the elucidation
of genetically determined developmental disorders is reducing this
uncertainty and replacing these generic labels with specific conditions.
The importance of labels is that they designate cause with increasing
accuracy. The increasing specificity of labels that should be permitted
to replace the generic labels of CP or SE results in increasing
accuracy of formulation of heritability, risks, prognosis, and treatment.7-9 Postnatal
neural development may impart a pseudoprogressive appearance to
the static deficits of CP, or of conditions within the wider spectrum
of SE, due to the fact that damage to various systems may not become
fully apparent until such systems “come on line” during
postnatal development.3,4,7 It is important to note that
despite the possible infantile worsening of manifestations, most
children with CP will experience, at varied rates and degrees, improvement
over the course of their development.3,8,9
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The incidence of CP is approximately 0.1% to 0.25% of
all live births, with little international variation among countries
with modern medical facilities.10,11 Modern neonatology
has greatly increased the survival of very low birth weight babies,
whose risk for CP is approximately eight-fold greater than for larger
neonates.12 Interestingly, increases in utilization ...