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A physiologic approach to neonatal resuscitation

Introduction Neonatal resuscitation is commonly defined as the assistance given to infants immediately after birth as they transition to newborn life. From a physiologic perspective, this transition involves some of the most complex and profound changes that any human will likely encounter during their life. The airways that are filled with liquid during fetal life must be cleared to allow the entry of air and onset…

Neonatal Neurology in the Low-Resource Setting for the Term and Preterm Newborn

Introduction Prominent identified causes of brain injury in the low-resource setting (LRS) include hypoxic-ischemic encephalopathy (HIE)/neonatal encephalopathy (NE), germinal matrix–intraventricular hemorrhage (GMH-IVH) in the premature infant, prolonged neonatal seizures, neonatal hypoglycemia, and hyperbilirubinemia (kernicterus). The magnitude of the contributions of these individual conditions to brain injury is underestimated in the LRS because of the lack of consistent standardized and repeated neurological assessments; neuroimaging (cranial ultrasound [CUS]…

Passive Addiction and Teratogenic Effects

Drugs can exert major effects on the developing central nervous system (CNS). In the broadest sense, drugs may disturb specific developmental events in the brain and, in turn, produce teratogenic effects. In addition, maternal ingestion of certain drugs can result in passive addiction of the fetus, and postnatally lead to a neonatal withdrawal or abstinence syndrome. The capacity for teratogenicity was first recognized in the late…

Brain Tumors and Vein of Galen Malformations

In this chapter, space-occupying lesions including brain tumors, vein of Galen malformations, and arachnoid cysts are discussed. These disorders are considered in the same chapter because they represent important intracranial mass lesions and share certain clinical features. We do not review in detail management, which is considered in depth in standard neurosurgical writings. Improvements in management in recent years have made the prognosis of these serious…

Injuries of Extracranial, Cranial, Intracranial, Spinal Cord, and Peripheral Nervous System Structures

INTRODUCTION This chapter is focused on injuries of extracranial, cranial, intracranial, spinal cord, and peripheral nervous system structures. In particular, the emphasis is on those disorders that appear to be related primarily to mechanical trauma. Adverse mechanical events occur principally during labor and delivery. Unfortunately, such events often lead to criticism of obstetrical management. Such criticism generally is unwarranted, because the mechanical factors are most often…

Bacterial and Fungal Intracranial Infections

Bacterial infections of the central nervous system (CNS) in the newborn are common and are of major clinical importance. By far the most frequent of these infections is neonatal bacterial meningitis, and this chapter deals with this disorder in detail. Other bacterial processes include primary intracranial infections (e.g., epidural and subdural empyema and brain abscess) and disorders in which involvement of the CNS is secondary to…

Viral, Protozoan, and Related Intracranial Infections

The central nervous system (CNS) and its covering membranes may become involved in a variety of infectious processes, with devastating effects on structure and function. Infections may occur during intrauterine development, in association with the birth process, or in the first postnatal days or weeks. Microbial organisms implicated include several viruses, a protozoan (Toxoplasma gondii) , a spirochete (Treponema pallidum) , and numerous bacteria and fungi.…

Muscle Involvement and Restricted Disorders

Muscle, the final component of the motor system, is the site of abnormalities with those essential clinical manifestations of hypotonia and weakness that unify the other disorders of the motor system (see Chapter 36 ). In this chapter, we deal with important myopathic disorders in the neonatal patient. In addition, certain restricted disorders of the motor system are reviewed. LEVEL OF THE MUSCLE Disorders of muscle…

Levels Above Lower Motor Neuron to Neuromuscular Junction

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 chapter and in Chapter 37 , we review disorders of the neonatal motor system according to the following specific anatomical levels: levels above the lower motor…

Arthrogryposis Multiplex Congenita

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 is a syndrome , not a disease entity, and is discussed in this section because, albeit frequently syndromic (associated with other anomalies), it can also be…

Evaluation, Special Studies

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 cerebral cortex to its termination in the muscle. This and the next three chapters are concerned with neuromuscular disorders. In this chapter, the motor system is…

Degenerative Disorders of the Newborn

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 appropriately in this series of chapters concerned with metabolic disorders. Indeed, clinical overlap of some of these degenerative disorders with some of the metabolic disorders discussed…

Organic Acids

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 be firmly entrenched in the medical literature. Carboxylic acids are the most important organoid acids. Their functional group is the carboxyl group. These compounds would include…

Amino Acids

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 causes of devastating disturbances of neurological development that are potentially treatable, and second, they provide insight into normal and abnormal brain metabolism. Disorders of AA metabolism…

Bilirubin and Brain Injury

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. The development of therapeutic measures, such as exchange transfusion, and of preventive measures, such as the use of anti-Rh immune globulin to prevent maternal sensitization, resulted…

Glucose

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 to the brain may be even more important. The major source of brain glucose is the blood supply; thus it is readily understood that serious encephalopathy…

Preterm Intraventricular Hemorrhage/Posthemorrhagic Hydrocephalus

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 larger forms of IVH and their attendant complications. Moreover, the major forms of brain injury of the premature infant occur most commonly in the context of…

Cerebellar Hemorrhage

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 of critically ill infants and the greater availability and application of magnetic resonance imaging (MRI). Early studies reported a prevalence of cerebellar hemorrhage as high as…

Intracranial Hemorrhage: Subdural, Subarachnoid, Subpial, Intraventricular (Term Infant), Miscellaneous

Intracranial hemorrhage in the neonatal period is an important clinical problem. Its importance relates to a relatively high frequency of occurrence, accompanied at times by serious neurological sequelae or even death. Over the last decade there have been changes in the relative frequency of intracranial hemorrhage due to changes in obstetrical practice, such as increased vacuum-assisted delivery and reduced rotational forceps, and the improved survival of…