In another group—acquired infantile hemiplegia—a normal infant or young child, usually between the ages of 3 and 18 months, develops a massive hemiplegia, with or without aphasia, within hours. The disorder often begins with seizures, and the hemiplegia may not be recognized until the seizures have subsided. In Banker’s series of autopsy cases, there was arterial or venous thrombosis in some cases, but instances without vascular occlusions were found. Some of the latter cases, in which arteriography had been normal, may have been embolic, possibly of cardiac origin. In the recent era, imaging has shown a large area of cerebral infarction, consistent with a stroke in the territory of the middle cerebral artery (Fig. 37-15). If the stroke occurs at an early age, the recovery of speech may be complete, though reduced scholastic capacity remains. The degree of recovery of motor function varies. Often, as the deficit recedes, the arm becomes involved by athetotic, tremulous, or ataxic movements; there may be an interval of months or years between the hemiplegia and the athetosis.
Destructive lesions underlie most of the cases of infantile hemiplegia and some cases of bilateral hemiplegia (as well as many cases of seizures in the first few days of life). The pathologic change is essentially that of ischemic necrosis. In many cases, the lesions must have been acquired in utero. Precipitant delivery, fetal distress, and prepartum uterine hemorrhage may have been indications, more so than causes, of the process. What is most notable is that the ischemia tends to affect the tissues lying in arterial cortical border zones; there may also be venous stasis with congestion and hemorrhage occurring particularly in the deep central structures such as the basal ganglia and periventricular matrix zones. If they are purely hypoxic, the lesions should be bilateral. Myers has reproduced such lesions in the neonatal monkey by reducing the maternal circulation for several hours. As the lesions heal, the monkeys develop the same gliotic changes in the cortex and white matter of the cerebrum (lobar sclerosis) and the “marbling” (état marbré) that characterizes the brains of patients with spastic diplegia and double athetosis (see below).
The quadriplegic state differs from bilateral hemiplegias in that the bulbar musculature is often involved in the latter and developmental delay is more severe. The condition is relatively rare and is usually a result of a bilateral cerebral lesion. However, one should also be alert to the possibility of a high cervical cord lesion. In the infant, this is usually the result of a fracture dislocation of the cervical spine incurred during a difficult breech delivery. Similarly, in paraplegia, with weakness or paralysis limited to the legs, the lesion may be either a cerebral or a spinal one. Sphincteric disturbances and a loss of somatic sensation below a certain level on the trunk always point to a spinal localization. Congenital cysts, tumors, and diastematomyelia are more frequently causes of paraplegia than of quadriplegia. Another recognized cause of infantile paraplegia is spinal cord infarction from thrombotic complications of umbilical artery catheterization.
This is a complex subject that has not been fully elucidated clinically or genetically. The concern is that persistently low levels of glucose will produce neuronal damage, as it has in experimental models. Its mildest form is a transient postpartum condition that occurs in premature and small for gestational age newborns and usually clears within hours. It is detected by a heel stick blood sample taken at birth and, if the glucose concentration is not below 45 mg/dL, often requires a repeat sample and confirmation by whole blood testing. Persistently low levels of glucose may be manifest as seizures or jitteriness.