Pathology Of Spasticity in Children Undergoing SDR

Pathophysiology

Preterm Infant

• PVL: PVL is the common pathology seen in cerebral palsy of the premature infant.  It is a white matter injury due to either ischemia/hypoxia or inflammation within the periventricular white matter.   IVH is a risk factor for its development but is a separate pathologic process and etiology for cerebral palsy.  The corticospinal pathway passes through the periventricular region and, as a consequence, is affected in the injury.
• IVH: IVH is the bleeding from the subependymal matrix into the ventricles seen in premature infants.  The smooth muscles of the brain’s arterioles do not develop until late in gestation resulting in this vulnerability.

Term Infant

• Gray matter injury: As the brain’s vascularity matures the injuries resulting from ischemia and hemorrhage begin to resemble those seen in adults.  Injuries tend to be in watershed areas and in the basal ganglia.  This gives rise to extrapyramidal injuries.  Dyskinetic CP becomes more common than the spastic CP of prematurity.

Molecular/Genetic Pathology

Cerebral Palsy

• Associated genetic conditions: Genetic anomalies related to production of proteins involved in inflammation or coagulation in the infant or vascular endothelium formation within the placenta (73).

Familial Spastic Parapareis

• 59 genes identified: To date there have been 59 genes identified as being associated with Familial Spastic Parapareis with 19 being AD,sa35 being AR and 5 being X-Linked (63).