Abstract
Diagnosis of congenital anomalies of the central nervous system has been made easily since the introduction of computed tomography. Clinical diagnosis of congenital anomalies must include determination of their treatablity and prognosis. For the management of congenital anomalies of the central nervous system, investigators should have standardized classification and criteria for their diagnosis. Classification of congenital anomalies of the central nervous system has usually been made by formative pathogenesis. From the clinical standpoint, classification based on developmental stage and causual pathogenesis is more useful. For this purpose, the classification advocated by Dr. DeMyer may fulfil the criterion. This classification is a dendrogram consisting of disorders in three stages; cytogenesis, histogenesis and organogenesis. Disorder of genes presents as metabolic abnormalities and does not show cerebral malformations. Disorder of chromosomes usually shows mental defects and microcephaly without obvious cerebral malformations. Disorder of histogenesis represented by neurocutaneous syndrome does not reveal malformations either. Since stages of development of the central nervous system are long in duration, from early stage of formation of the neural tube to the perinatal period, disorder of organogenesis develops various types of cerebral malformation. In general, the earlier the disorder occurs, the more severe is the malformation. In the present communication, discussion is made to lead to standardization of nomenclature of malformations of the central nervous system.
