bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acute Ischemic Stroke Evaluation and Treatment
Authors: Scharf Eugene L. 
Published: November 2021

Abstract
Acute ischemic stroke is a neurologic emergency where an estimated 2 million neurons a minute are lost secondary to ischemia. Treatments of acute stroke are directed at early revascularization of the occluded vessel and to preserve neuronal death and improve collateral flow. Treatments are time sensitive, an aspect that places great importance on early symptom recognition, correct diagnosis, and clinical management. In acute ischemic stroke, in short, “time is brain.”
Secondary Prevention of Ischemic Stroke
Authors: Williams Lindsy N. 
Published: November 2021

Abstract
After an ischemic stroke is diagnosed, a diagnostic evaluation ensues to determine the mechanism of the stroke and contributing risk factors. The appropriate antithrombotic is selected in accordance with the mechanism of the stroke, and the contributing risk factors are treated with pharmacologic agents and lifestyle changes. This chapter discusses the selection of antithrombotic medication, medical treatment, and lifestyle changes for contributing risk factors.
Intraparenchymal Cerebral Hemorrhage
Authors: Aguilar Maria I. 
Published: November 2021

Abstract
Intraparenchymal cerebral hemorrhage (ICH) is the presence of blood in the brain parenchyma. It is a neurologic emergency and may carry severe morbidity and death. This chapter focuses mainly on spontaneous, nontraumatic ICH (ie, hemorrhage not related to trauma, arteriovenous malformation, cerebral aneurysm, or tumor). ICH accounts for 15% to 20% of all new strokes annually. Among the US general population, the incidence is 15 cases per 100,000 person-years.
Unruptured Intracranial Aneurysms and Vascular Malformations
Authors: Brown Robert D. 
Published: November 2021

Abstract
Knowledge of the natural history of unruptured intracranial aneurysms and vascular malformations of the brain is important because this information can be weighed against the morbidity and death rates of intervention to decide on the most appropriate treatment of an individual patient. This chapter reviews the epidemiologic factors and natural history of common intracranial vascular abnormalities.
Principles and Management of Increased Intracranial Pressure
Authors: Kumar Shivram , Wijdicks Eelco F. M. 
Published: November 2021

Abstract
Intracranial pressure (ICP) is a reflection of the total volume inside the skull. Normal ICP is 5 to 15 mm Hg. Intracranial hypertension is defined as sustained ICP of more than 20 mm Hg. Increased ICP may lead to a reduction of cerebral perfusion pressure, a shift of brain tissue, and, as a result, secondary brainstem injury. Early recognition and treatment of elevated ICP are needed to prevent irreversible damage.
Nontraumatic Subarachnoid Hemorrhage
Authors: Chakraborty Tia , Fugate Jennifer E. 
Published: November 2021

Abstract
Subarachnoid hemorrhage (SAH) is defined as blood in the subarachnoid space. Nontraumatic SAH is most commonly caused by rupture of an aneurysm located at the circle of Willis. Patients often present with acute thunderclap headache but also may lose consciousness or have focal neurologic deficits. Detection of an aneurysm, if present, and its treatment are needed urgently to prevent recurrent bleeding. Patients with nontraumatic SAH are prone to numerous complications that require preventative measures, early recognition, and treatment.
Acute Hyperthermia Syndromes
Authors: Hawkes Maximiliano A. , Wijdicks Eelco F. M. 
Published: November 2021

Abstract
A high core body temperature is a medical emergency. Neuronal mitochondria and plasma membranes are thought to undergo protein changes at temperatures higher than 40°C, which lead to brain damage or dysfunction. In addition, the effects of hyperthermia on an already injured brain cause further injury. Control of fever is one of the important additional interventions necessary to control increased intracranial pressure and status epilepticus and to treat hypertensive surges in patients with sympathetic system overdrive.
Treatment of Multiple Sclerosis
Authors: Kantarci Orhun H. 
Published: November 2021

Abstract
A comprehensive and ideal approach to treatment of multiple sclerosis (MS) has 5 levels of intervention. They are 1) prevention of active disease (clinical or subclinical relapses) in the first place, to prevent cumulative relapse-associated disability burden; 2) shortening the duration of active inflammatory demyelination associated with relapses, to limit the extent of irreversible central nervous system injury; 3) induction of more complete recovery after a relapse, to prevent or delay an ensuing chronic progressive neurodegeneration; 4) slowing the progressive phase of MS after progression ensues; and 5) treatment of symptoms and complications. The ultimate combined goal of these intervention levels is to alter the natural history of MS and prevent progression responsible for the main quantity of disability in MS, thereby improving the quality of life for patients with MS. Currently, treating physicians can accomplish levels 1, 2, and 5. Level 3 and level 4 interventions are yet to be developed sufficiently to affect the disease course meaningfully.
Non–Multiple Sclerosis Central Nervous System Inflammatory Disease
Authors: Zalewski Nicholas L. , Pittock Sean J. 
Published: November 2021

Abstract
Multiple sclerosis (MS) is the most common inflammatory central nervous system (CNS) disease. Yet, clinicians should be cognizant of other demyelinating and nondemyelinating CNS inflammatory diseases, some of which mimic MS. This chapter reviews the spectrum of non-MS CNS inflammatory diseases and MS mimics.
Classification and Approach to Movement Disorders
Authors: Youssef Paul E. , Mack Kenneth J. , Flemming Kelly D. 
Published: November 2021

Abstract
Movement disorders are conventionally divided into 2 major categories. Hyperkinetic movement disorders (also called dyskinesias) are excessive, often repetitive, involuntary movements that intrude into the normal flow of motor activity. This category includes chorea, dystonia, myoclonus, stereotypies, tics, and tremor. Hypokinetic movement disorders are akinesia (lack of movement), hypokinesia (reduced amplitude of movement), bradykinesia (slow movement), and rigidity. Parkinsonism is the most common hypokinetic movement disorder. In childhood, hyperkinetic disorders are common, whereas hypokinetic movement disorders are relatively uncommon.
Hypokinetic Movement Disorders: Parkinson Disease
Authors: Tisel Sarah M. , Klassen Bryan T. 
Published: November 2021

Abstract
Parkinson disease (PD) is the classic hypokinetic movement disorder and one of the most common and widely recognized neurodegenerative conditions. PD is distinct from parkinsonism, a term that refers to a syndrome of rest tremor, bradykinesia, rigidity, and postural instability. The mechanism behind the progressive degeneration and cell death that result in PD is not precisely understood. Substantia nigra depigmentation occurs on a macroscopic level and loss of dopaminergic neurons and gliosis on a microscopic level.
Cerebellar Disorders and Ataxias: Acquired Disorders
Authors: Hassan Anhar 
Published: November 2021

Abstract
Disorders of the cerebellum or its connections can result in ataxia characterized by imbalance and incoordination of gait, limbs, speech, and eye movements. The pathologic changes may be confined to the cerebellum or simultaneously affect other parts of the central nervous system or peripheral nervous system. Ataxias are generally classified as acquired, inherited, or sporadic. The differential diagnosis is broad and daunting. However, a detailed history and examination can rapidly narrow the list. Key information includes age at onset; rate of disease progression; family history; presence of pure cerebellar syndrome or other neurologic signs; other systemic features; and imaging findings.
Cerebellar Disorders and Ataxias: Inherited Disorders
Authors: Hassan Anhar 
Published: November 2021

Abstract
Disorders of the cerebellum or its circuitry can result in ataxia. These disorders may be acquired or inherited. Inherited ataxias may be autosomal recessive (eg. Friedrich ataxia), autosomal dominant (eg, spinal cerebellar atrophy) or X linked (eg, fragile X–associated ataxia syndrome). Chapter 73 (“Cerebellar Disorders and Ataxias: Acquired Disorders”) reviews the clinical approach to patients with ataxia and discusses acquired forms of ataxia. This chapter reviews clinical approaches, diagnostic details, and treatment of inherited ataxias.
Syndromes of Cognitive Dysfunction
Authors: Kumar Shivram , Flemming Kelly D. 
Published: November 2021

Abstract
Cognitive function refers to the mental process of knowing things. It includes high-level cortical functions such as memory, language, perception, and executive function (planning, initiating, and reasoning) that generally depend on the alert state and focused attention. Dysfunction of cognition without a change in consciousness may result in various disorders, including aphasia, apraxia, agnosia, executive dysfunction, and memory disorders such as dementia and amnesia. Transient dysfunction of cognition associated with a change in level of consciousness or attention may be due to delirium or confusional states. This chapter broadly introduces the specific definitions of cognitive dysfunction and the overall differential diagnoses.
Nondegenerative Dementias and Encephalopathies
Authors: Flanagan Eoin P. 
Published: November 2021

Abstract
Nondegenerative dementias are a diverse but important group of cognitive disorders because they may be reversible with treatment. Thus, evaluation is important when a nondegenerative dementia is suspected. Many causes of nondegenerative dementia result in what is known as subcortical dementia, which is thought to be primarily due to damage to the frontal subcortical connections. Typical clinical features include inattention, bradyphrenia (slowed thought process), executive dysfunction (difficulties planning and sequencing tasks), apathy, psychomotor slowing, and mood disorders. Gait apraxia and urinary difficulties may coexist. Cortical features such as agnosia, seizures, aphasia, and ideomotor apraxia are typically absent.
Epidemiology and Pathophysiology of Epilepsy
Authors: Burkholder David B. 
Published: November 2021

Abstract
About 10% of people in the United States have 1 seizure in their lifetime; less than 4% have recurrent seizures or epilepsy. Currently, more than 3.4 million people in the United States have active epilepsy, making it one of the most common neurologic disorders. Seizures can develop at any age, but the most common times are during childhood and after age 60 years. The greatest incidence is in elderly patients. Acute symptomatic seizures (also called provoked seizures or reactive seizures) result from new and active insults to the central nervous system.
Treatment of Epilepsy
Authors: Hoerth Matthew T. 
Published: November 2021

Abstract
A systematic approach can simplify the process of epilepsy treatment, and knowledge of the antiseizure drugs is essential. This chapter summarizes the approach to treatment, presents a summary of medications and alternative therapies, and discusses treatment in special situations. A rational approach should be taken when selecting medications for the treatment of seizures associated with epilepsy. The choices for drug selection are ever expanding, which can make the selection process difficult for even experienced epileptologists, let alone trainees and patients. On the other hand, the diverse choices available allow medications to be tailored to each patient’s specific situation.
Peripheral Nerve Disorders
Authors: Berini Sarah E. , Staff Nathan P. 
Published: November 2021

Abstract
Disorders of the peripheral nerves are some of the most common conditions that neurologists face in clinical practice. The wide differential diagnosis that often accompanies peripheral nerve disorders may be narrowed by careful attention to the history (time course, severity, preexisting disease, and family history); peripheral neuroanatomy; patient symptomatology (sensory loss, paresthesia, pain, and weakness); and neurologic examination (sensory loss, weakness, atrophy, and reduced muscle stretch reflexes). Electromyography is used to assess large-diameter myelinated axons (touch, pressure, vibration, proprioception, and motor) and aid in localization by helping to narrow the differential diagnosis and predict axonal or demyelinating pathophysiology.
Neuromuscular Junction Disorders
Authors: Goodman Brent P. 
Published: November 2021

Abstract
The neuromuscular junction (NMJ) is a critical component of the motor unit that is made up of the distal, unmyelinated nerve terminal, synaptic space, and end-plate region of the muscle fiber. Contraction of muscle fiber involves a coordinated series of steps that ultimately generates an action potential at the muscle end plate (also known as an end-plate potential). Normally the end-plate potential substantially exceeds the threshold necessary to trigger an action potential in the muscle fiber, and this difference is termed the safety factor of neuromuscular transmission. Disorders that affect the NMJ reduce this safety factor, a change that results in fatigable weakness.
Autonomic Disorders
Authors: Singer Wolfgang 
Published: November 2021

Abstract
A practical but arbitrary classification of autonomic disorders is used at Mayo Clinic. Presented in a modified and abbreviated form in this chapter, that classification serves to organize and provide an overview of autonomic disorders. It is organized by anatomical systems and levels, although some syndromes and disorders do not fit neatly into this organization and therefore are assigned their own category. This classification quickly shows how abundant and interspersed autonomic dysfunction is among various categories of neurologic disease. Therefore, the role of this chapter is not to describe every disorder that can be associated with autonomic dysfunction. Rather, the chapter focuses on disorders with autonomic dysfunction as a predominant or important feature and thereby covers what are generally referred to as autonomic disorders.
Clinical Neurotology
Authors: Eggers Scott D. 
Published: November 2021

Abstract
Normal balance is the consequence of continuous interaction between visual, vestibular, and proprioceptive mechanisms. The vestibular system is a system in which head movements and positions cause mechanical forces to be transduced into electrochemical signals that are relayed to the central nervous system for the purpose of maintaining clear stable vision and equilibrium. With a basic understanding of physiology, common disease processes, and examination techniques, a correct diagnosis can generally be made at the bedside.
Primary Headache Disorders: Migraine, Tension-Type, and Chronic Daily Headaches
Authors: Whealy Mark A. , Cutrer F. Michael 
Published: November 2021

Abstract
Primary headache disorders are those in which the headache is the primary feature of the disorder and is not otherwise explained by a structural, genetic, or metabolic cause. This chapter reviews the clinical features, diagnosis, and treatment of common primary headache disorders, including migraine, tension-type, and chronic daily headaches. Migraine usually starts during adolescence, and 90% of patients with migraine have their first attack by age 40 years. In childhood, there is a slight male predominance, but after puberty, a 3:1 female predominance is established. The highest reported prevalence of migraine is in patients who are 30 to 39 years old.
Primary Headache Disorders: Trigeminal Autonomic Cephalalgias, Headaches With Specific Triggers, and Other Primary Headache Disorders
Authors: VanderPluym Juliana H. , Halker Singh Rashmi B. 
Published: November 2021

Abstract
Primary headache disorders are those in which the predominant clinical feature is headache that is not caused by or attributed to another disorder. This chapter reviews the common primary headache disorders of migraine, tension-type headache, and chronic daily headache. The present chapter reviews less common primary headache disorders, such as trigeminal autonomic cephalalgias and headaches with certain triggers. A careful history, physical examination, and additional testing are often necessary to rule out a secondary cause.
Secondary Headache Disorders
Authors: Starling Amaal J. , Dodick David W. 
Published: November 2021

Abstract
In the evaluation of a patient with headache, the first task is to differentiate between a secondary headache and a primary headache. This step is essential because secondary causes of headache may require vastly different evaluation and treatment than primary headache disorders. Thunderclap headache (TCH) is an acute, severe headache with an abrupt onset, reaching maximum intensity in less than 1 minute. TCH is a neurologic emergency and should immediately prompt an urgent evaluation for a secondary headache.
Neuropathic Pain Syndromes
Authors: Watson James C. 
Published: November 2021

Abstract
The International Association for the Study of Pain defines neuropathic pain as pain that is initiated or caused by a lesion or disease affecting the somatosensory system in either the peripheral nervous system or the central nervous system. Several well-recognized descriptors for neuropathic pain suggest a neuropathic rather than nociceptive pathophysiology (hot, burning, painful cold, freezing, prickling or tingling, pins and needles, electrical, shooting, stabbing, lancinating, and itching). However, whether the pain descriptors are used alone or incorporated into questionnaires to identify neuropathic pain, their sensitivity and specificity are limited (generally 70%-85%); therefore, verbal pain descriptors are insufficient for making the diagnosis of neuropathic pain.
Introduction to Neoplastic Disease
Authors: Porter Alyx B. 
Published: November 2021

Abstract
The advanced imaging readily available at most medical centers allows easier identification of neoplastic disease of the nervous system. Despite earlier detection, nervous system neoplasms continue to be an important cause of neurologic morbidity and death. This chapter broadly reviews the clinical manifestations and general approach to diagnosis and management of nervous system tumors. Subsequent chapters review individual types of tumors, complications of therapy for tumors, and paraneoplastic diseases.
Glial Tumors
Authors: Sharma Akanksha , Johnson Derek R. 
Published: November 2021

Abstract
Glial tumors are primary brain and spinal cord tumors arising from supporting cells of the central nervous system, including oligodendrocytes, astrocytes, microglia, and ependymal cells. Collectively, glial tumors are the most common category of primary brain tumors. They may be infiltrative or well circumscribed. Prognosis ranges from surgical cure to survival of less than 2 years, depending on tumor type. Age is the strongest risk factor for glioma in adults, with incidence rates increasing from a low of 3.2 per 100,000 person-years among persons age 20 to 34 years to a high of 19.3 per 100,000 person-years among those age 75 to 84 years.
Peripheral Nerve Sheath Tumors
Authors: Kumar Shivram , Ruff Michael W. 
Published: November 2021

Abstract
Nerve sheath tumors involve the connective tissue surrounding a peripheral nerve. They may be found incidentally or may be symptomatic. In most cases, nerve sheath tumors are benign. A detailed personal and family history and careful physical examination should be performed to assess for association with neurofibromatosis 1 or 2 or schwannomatosis. This chapter reviews the genetics, clinical features, diagnosis, and management of the common types of peripheral nerve sheath tumors.
Metastatic Disease
Authors: Sharma Akanksha , Porter Alyx B. 
Published: November 2021

Abstract
Intracranial metastases are the most common brain tumors in adults and outnumber primary brain tumors by 10 to 1. Nearly 200,000 patients are affected every year in the United States, and these metastases have become the most common neurologic complication of cancer. Intracranial metastases are occurring with increased frequency as a result of improved therapies for systemic cancers that extend survival. Most patients with brain metastases have lung cancer as the primary malignancy, followed in frequency by breast, melanoma, renal cell, and thyroid cancers. The prognosis when brain metastases are detected differs on the basis of the underlying cancer type.
Paraneoplastic and Other Autoimmune Neurologic Disorders
Authors: Lopez Chiriboga A. Sebastian , McKeon Andrew 
Published: November 2021

Abstract
A paraneoplastic disorder arises from remote effects of malignancy, rather than direct tumor invasion. Paraneoplastic neurologic disorders occur because of the vigorous immune responses directed against antigens expressed in tumors. The vigor of the immune response usually ensures that neoplasm is confined to the primary organ and regional lymph nodes. However, in the clinical setting of paraneoplastic disease, the immune response may attack any part of the nervous system (brain, spinal cord, nerve, neuromuscular junction, or muscle), and antibodies are either directed at intracellular neuronal proteins or against neuronal cell surface or synaptic proteins. Treatment is often aimed at the cancer and suppression of the abnormal immune response.
Syndromic Approach to Neuroinfectious Diseases
Authors: Kumar Shivram , Flemming Kelly D. 
Published: November 2021

Abstract
A general approach to clinical syndromes associated with infection of the nervous system can be useful when an infectious agent is suspected. This chapter provides a general overview of clinical syndromes of neurologic infectious disease. Subsequent chapters review bacterial, viral, fungal, and protozoan infections and prion disorders. Meningitis refers to inflammation of the leptomeninges caused by either infectious or noninfectious processes. It is associated with an increased white blood cell count in the cerebrospinal fluid. Infectious agents may be bacteria, viruses, or fungi. Noninfectious causes include autoimmune conditions, malignancies, and medications.
Bacterial Infections of the Nervous System
Authors: Toledano Michel 
Published: November 2021

Abstract
Bacterial infections of the nervous system manifest themselves in several ways, most of which are neurologic emergencies. This chapter reviews the classic syndromes of meningoencephalitis and focal infection (cerebritis or abscess) and the syndromes of neurosyphilis, tuberculosis, botulism, leprosy, Lyme disease, Whipple disease, rickettsiosis, diphtheria, and ventricular shunt infections.
Neurocutaneous Disorders
Authors: Keating Gesina F. 
Published: November 2021

Abstract
Neurocutaneous disorders, formerly called phakomatoses, are characterized by cutaneous and neurologic findings. Many are genetic, but some are sporadic. Often these disorders affect other organ systems as well and require lifetime surveillance for complications. Neurofibromatosis type 1 is the most common neurocutaneous disorder. With a prevalence of approximately 1 in 3,000 persons, it is more than 10 times more common than neurofibromatosis type 2.
Lysosomal Storage Disorders
Authors: Dhamija Radhika , Conboy Erin , Wong-Kisiel Lily C. 
Published: November 2021

Abstract
Lysosomes are membrane-bound organelles that degrade various macromolecules. Lysosomal storage diseases are a clinically, enzymatically, and genetically heterogeneous group of disorders resulting from intracellular accumulation of substrates. Mechanisms of lysosomal storage disorders include 1) primary deficiency of specific hydrolases; 2) defects in activator proteins required for enzyme-substrate interactions in posttranslational modification of enzymes or in transport of the substrate from lysosomes; and 3) abnormalities of fusion between autophagic vacuoles and lysosomes. Substrate accumulation is slowly progressive, leading to considerable morbidity and mortality.