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Anoxic-Ischemic Encephalopathy

When the heart stops and cerebral blood flow is interrupted during a cardiac arrest, patients lose consciousness and may remain comatose after resumption of circulation. Such a global injury to the brain is understandably profound, and more than 70% of patients die or remain comatose 24 hours after cardiopulmonary resuscitation (CPR) ( ). Anoxia describes the complete lack of oxygen delivery (e.g., complete cessation of blood…

Autoimmune Encephalitis with Antibodies to Cell Surface Antigens

Autoimmune encephalitis with antibodies to neuronal cell surface/synaptic antigens (further referred to as autoimmune encephalitis) are a group of neuropsychiatric disorders ( Table 82.1 ) in which the antibodies produce neuronal dysfunction by direct interaction with their target antigen ( ). These disorders can occur with and without a cancer association and while they affect individuals of all ages, some syndromes preferentially affect young adults and…

Paraneoplastic Disorders of the Nervous System

Pathogenesis Paraneoplastic neurological syndromes (PNSs) are a heterogeneous group of disorders that are immune mediated ( Table 81.1 ). It is postulated that the expression of neuronal proteins (called onconeuronal proteins) by a tumor provokes an immune response that is misdirected against the nervous system. This hypothesis is supported by the detection in the serum and cerebrospinal fluid (CSF) of anti-neuronal antibodies that react with antigens…

Multiple Sclerosis and Other Inflammatory Demyelinating Diseases of the Central Nervous System

Diseases affecting central nervous system (CNS) myelin can be classified on the basis of whether a primary biochemical abnormality of myelin exists (dysmyelinating) or whether some other process damages the myelin or oligodendroglial cell (demyelinating) . Demyelinating diseases in which normal myelin is disrupted include autoimmune, infectious, toxic and metabolic, and vascular processes. Dysmyelinating diseases in which a primary abnormality of the formation of myelin exists…

Bacterial, Fungal, and Parasitic Diseases of the Nervous System

Prior to the modern antimicrobial era, non-viral infections of the central nervous system (CNS) were almost universally fatal. The diagnosis and treatment of CNS infections have improved significantly with advancements made in spinal fluid diagnostic tests, neuroimaging, antimicrobial therapy, and neurosurgical techniques. Despite these advancements, diagnosing CNS infections can be difficult, and a delay in diagnosis and treatment can lead to increased morbidity and mortality. Thus,…

Viral Encephalitis and Meningitis

Hundreds of viruses exhibit tropism for the central (CNS) and/or peripheral (PNS) nervous systems. In the case of some viruses, involvement of the CNS or PNS is the predominant feature of illness, whereas in others involvement of the nervous system is a rare complication of a more generalized illness. Viral infection of the nervous system can result in a myriad of clinical presentations occurring separately or…

Neurological Manifestations of Human Immunodeficiency Virus Infection in Adults

Epidemiology and Current Trends Acquired immunodeficiency syndrome (AIDS) was first recognized in the United States in the summer of 1981 when unexplained occurrences of Pneumocystis jirovecii (formerly carinii ) pneumonia and Kaposi sarcoma were reported in cohorts of previously healthy homosexual men in Los Angeles, New York, and San Francisco. Within months, the disease became recognized in intravenous drug users (IDUs) and soon thereafter in recipients…

Nervous System Metastases

Brain Metastases Epidemiology Parenchymal brain metastases (BMs) are the most common direct neurological complication of systemic cancer. BMs are the most common intracranial tumor in adults, with 200,000–300,000 diagnosed annually in the United States ( ). In comparison, there are 35,000 new patients with primary brain tumors diagnosed each year in the United States. The precise incidence of BM is unknown and likely much higher, likely…

Primary Nervous System Tumors in Infants and Children

Pediatric Primary Nervous System Tumors Primary brain tumors account for nearly 20% of all malignancies during childhood and adolescence worldwide, with an age-standardized incidence rate of 28.2 per million persons ( ; ). These tumors are second only to leukemia in frequency among all childhood cancers and are the most common solid tumor in children ( ; ). The location, histological features, prognosis, and treatment of…

Primary Nervous System Tumors in Adults

In the year 2019, approximately 86,970 cases of primary nervous system tumors are expected to occur in the United States ( ). The World Health Organization (WHO) distinguishes between six groups of neoplasms. Meningeal tumors (mostly meningioma) and neuroepithelial tumors (astrocytic tumors including glioblastoma multiforme [GBM], oligodendrogliomas, and others) each account for about one-third of nervous system neoplasms. The remaining one-third is composed of tumors of…

Clinical Features of Brain Tumors and Complications of Their Treatment

Clinical Features of Brain Tumors Overview of Symptoms Patients with both primary and metastatic brain tumors can present with a variety of clinical symptoms and neurological signs. These manifestations are not specific for individual tumor subtypes or brain tumors, and symptoms can easily be mistaken for a number of other neurological conditions. In general, symptoms and signs are influenced by the underlying tumor’s location, size, and…

Pathology and Molecular Genetics

Acknowledgments Michael Jansen, Arie Perry, Reid R. Heffner, Jr., and David N. Louis were authors of this chapter in the previous edition. General Principles of Nervous System Tumor Biology Tumors of the nervous system, like other human neoplasms, are dysregulated clonal proliferations that arise secondary to changes in cellular genetics and physiology. In many cases, alterations involving growth-promoting oncogenes, growth-checking tumor suppressors, and cell death genes…

Epidemiology of Brain Tumors

Primary brain tumors are a diverse group of neoplasms arising from different cells of the central nervous system (CNS), including neuroepithelial tissue, the meninges, and cranial and spinal nerves. Within each of these cell types, tumors can exhibit distinct pathological characteristics, biological behavior, and clinical outcomes, leading to the complex classification of more than 100 different subtypes of brain tumors ( ). Tumors originating from glial…

Central Nervous System Vasculitis

Central nervous system vasculitis (CNSV), alternatively termed primary angiitis of the central nervous system (PACNS) or granulomatous angiitis of the central nervous system, is a rare diagnosis with an estimated incidence rate of 2.4 cases per 1 million person years ( ). The diagnosis can be challenging given the nonspecific presenting signs of headache, encephalopathy, and variable focal neurological signs. Here we outline some of the…

Spinal Cord Vascular Disease

The spinal cord is subject to many of the same vascular diseases that involve the brain, but its unique anatomy and embryology render it susceptible to some syndromes that do not have intracranial counterparts. Although some vascular myelopathic processes have classic syndromic presentations, many may be more challenging to differentiate from other forms of myelopathy. Ischemic diseases of the spinal cord have been well characterized; however,…

Stroke in Children

Stroke and the Developing Cerebrovascular System Unlike adults, in most children with stroke, conditions such as diabetes and hypertension make little contribution to the etiology of stroke. Developmental, genetic, and environmental factors are the major contributors to cerebrovascular injury in children. Epidemiology Full-Term and Near-Term Neonates Neonates appear to be at higher risk for stroke than older children. Asymptomatic subdural hemorrhage affects almost half of term…

Intracranial Aneurysms and Subarachnoid Hemorrhage

An intracranial aneurysm is a cerebrovascular disorder in which weakness in the wall of a brain artery causes dilation or ballooning, which can grow and rupture over time. The term aneurysm originally comes from the Greek aneurysma—ana meaning “across” and eurys meaning “broad.” Rupture of an intracranial aneurysm causes aneurysmal subarachnoid hemorrhage (aSAH), which accounts for 5%–10% of all strokes. aSAH causes greater morbidity and mortality…

Intracerebral Hemorrhage

Intracerebral hemorrhage (ICH) accounts for approximately 10%–20% of strokes. Its clinical importance derives from its high frequency and 30-day mortality, which is close to 50%. The incidence of ICH has remained stable in the past 3 decades ( ), despite a gradually improved level of detection and treatment of hypertension, suggesting that ICH due to other mechanisms, such as anticoagulant use and cerebral amyloid angiopathy (CAA),…

Ischemic Cerebrovascular Disease

Epidemiology and Risk Factors There are approximately 795,000 new or recurrent strokes annually in the United States (610,000 being first events and 185,000 being recurrent events) ( ) Despite declining stroke incidence, 7 million Americans above the age of 20 are estimated to have experienced a stroke. ( ). Approximately 88% of these strokes are ischemic and 8%–12% of ischemic strokes result in death within 30…

Trauma of the Nervous System: Peripheral Nerve Trauma

Historical interest in peripheral nervous system (PNS) trauma dates back many centuries. Leonardo Da Vinci made detailed anatomical drawings of the brachial plexus, believing this complex of nerves existed to ensure continued function of the upper extremity should one of its elements be severed (e.g., by a sword thrust) ( ). By 1885, Duchenne, Erb, and Klumpke all had recorded their landmark descriptions of various brachial…