An effective means of attaining an understanding of the major disorders of the neonatal motor system is to organize the approach to these disorders on the basis of the major affected anatomical site within the motor system. Thus in this…
Arthrogryposis multiplex congenita refers to a syndrome, apparent at birth, characterized by fixed positions of multiple joints and an associated limitation of movement. The term arthrogryposis is derived from the Greek and literally means bent joint . Arthrogryposis multiplex congenita…
Neuromuscular disorders may cause dramatic disability in the neonatal period. The dominant features of these disorders are muscle weakness and hypotonia. In this context, I consider neuromuscular disorders those that predominantly involve the motor system, from its origins in the…
Certain degenerative disorders of the developing nervous system may be clinically manifested in the neonatal period. Because most of these disorders are related to a disturbance in the metabolism of a lipid or some other compound, they are discussed most…
A series of metabolic disorders with prominent neurological accompaniments and serious deleterious effects on the developing central nervous system has been described under the designation organic acid disorders . The term organic acid is particularly imprecise but, unfortunately, appears to…
Since the late 1950s, numerous disorders of amino acid (AA) metabolism have been described with major implications for the developing nervous system. Although each of the disorders is rare, collectively they are important for two major reasons. First, they represent…
An important relationship between bilirubin and injury to the neonatal central nervous system (CNS) has been recognized for many years. The first comprehensive description of the most overt form of bilirubin encephalopathy (i.e., kernicterus) was provided by Schmorl in 1903.…
Introduction Glucose, like oxygen, is of essential and fundamental importance for brain metabolism. Indeed, because oxygen consumption is relatively low in the neonatal human brain and minimal in such areas as cerebral white matter (see Chapter 16 ), glucose supply…
Germinal matrix–intraventricular hemorrhage (GMH-IVH) is the most common variety of neonatal intracranial hemorrhage and is characteristic of the premature infant. The importance of the lesion relates not only to its high incidence but also to the essential gravity of the…
Cerebellar hemorrhage has been increasingly detected in the fetus and the preterm and full-term newborn in recent years. Currently, our understanding of cerebellar hemorrhagic injury and its long-term neurological sequelae has changed significantly, in large part because of increasing survival…