CEREBRAL DYSGENESIS (AGENESIS)

The term cerebral agenesis is frequently used by pediatricians and neurologists to denote a common entity characterized by mental defect plus developmental retardation associated with neurologic signs of varying degree. Congenital anomalies other than those of the central nervous system are also frequently associated with this condition. Cerebral agenesis represents a group of encephalopathies distinguishable in most instances from conditions produced by injury at birth; the lesions observed in cases of agenesis probably may be attributed to maldevelopment of the central nervous system in utero. In Alpers and Marcovitz'¹ excellent discussion of the subject, the pathologic evidence for intrauterine maldevelopment is reviewed to date.

Cerebral agenesis is, strictly speaking, a misnomer, since agenesis presupposes lack of development or nondevelopment of an organ or of part of an organ (as, for example, in the designation agenesis of the corpus callosum). Abnormal development of the cortex or of other parts of