Chapter 17 Acute and chronic viral infections
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Nikolaus Deigendesch, Werner Stenzel A large number of viruses belonging to various families are able to cause central nervous system (CNS) infections and contribute significantly to burden of disease in humans globally. Most viral CNS infections are benign and self-limiting, and most remain undiagnosed. However, some viruses can cause severe inflammation, leading to morbidity and mortality, and result in severe long-term residual damage and neurologic dysfunction in patients. The potential of viruses to cause CNS inflammation greatly v...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 16 Acute and chronic bacterial infections and sarcoidosis
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Nikolaus Deigendesch, Werner Stenzel Pathogen-induced inflammatory diseases mostly involving, but sometimes exclusively affecting, the central nervous system (CNS) manifest with a plethora of signs and symptoms. The different diseases are still difficult to cure despite modern diagnostic, preventive, and therapeutic advances, and this is mainly due to the host inflammatory response, leading to irreversible tissue damage. Knowledge about underlying pathomechanisms is constantly growing, and numerous studies on humans, human material, and...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 15 Neuropathology of epilepsy
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Eleonora Aronica, Angelika Mühlebner Epilepsy is one of the most common neurologic disorders, affecting about 50 million people worldwide. The disease is characterized by recurrent seizures, which are due to aberrant neuronal networks resulting in synchronous discharges. The term epilepsy encompasses a large spectrum of syndromes and diseases with different etiopathogenesis. The recent development of imaging and epilepsy surgery techniques is now enabling the identification of structural abnormalities that are part of the epileptic net...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 14 Neurotoxicology and drug-related disorders
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Serge Weis, Andreas Büttner Neuropsychiatric disorders caused by toxic substances pose a great diagnostic challenge due to the large variety of changes caused in the central and peripheral nervous system. The pathogenetic mechanisms at work are multifaceted and partly not solved. In human drug abusers (cannabis, opiates, cocaine, amphetamines, methamphetamine and “designer drugs”), a broad spectrum of central nervous system alterations are observed including infarction, intracerebral and subarachnoidal hemorrhage, hypoxic-ischemic ...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 13 Alcohol-related diseases
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Serge Weis, Andreas Büttner Alcohol abuse and dependence are serious medical and economic problems in Western countries. Brain changes encountered in alcoholism are manifold and encompass brain atrophy, selective neuronal loss, astroglial, and microglial changes. Alcohol-related disorders are complex multifactorial disorders where the interaction of multiple genes and environment plays an important role in the pathogenesis. (Source: Handbook of Clinical Neurology)
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 12 Nutritional and systemic metabolic disorders
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Serge Weis, Andreas Büttner Vitamin deficiency disorders display a wide variety of neurologic signs and symptoms, the pathogenesis of which is not clearly understood. Metabolic encephalopathies (hepatic, hypoglycemic, and uremic) have to be considered in the differential diagnosis of patients with cognitive impairment, motor disturbances, psychiatric symptoms, seizures, and neuropathies. Calcifications (vascular wall and parenchymal) occur in the normal aging brain and in neurodegeneration; some associated genes are already described. ...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 11 Neurodegeneration with brain iron accumulation
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Sarah Wiethoff, Henry Houlden Neurodegeneration with brain iron accumulation (NBIA) describes a heterogeneous group of inherited rare clinical and genetic entities. Clinical core symptoms comprise a combination of early-onset dystonia, pyramidal and extrapyramidal signs with ataxia, cognitive decline, behavioral abnormalities, and retinal and axonal neuropathy variably accompanying these core features. Increased nonphysiologic, nonaging-associated brain iron, most pronounced in the basal ganglia, is often termed the unifying characteris...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 10 Mitochondrial diseases
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Maria J. Molnar, Gabor G. Kovacs Mitochondrial disorders represent a major challenge in medicine. Most of the mitochondrial proteins are encoded by the nuclear DNA (nDNA), whereas a very small fraction is encoded by the mitochondrial DNA (mtDNA). Mutations in mtDNA or mitochondria-related nDNA genes can result in mitochondrial dysfunction. The disease usually affects multiple organs in varying locations and severity; however, there are some forms which affect a single organ. The diagnosis of mitochondrial disorders is based on clinical ...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 9 Neurometabolic and neurodegenerative diseases in children
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Josefine Radke, Werner Stenzel, Hans Hilmar Goebel Neurometabolic and neurodegenerative diseases in children (NDDC) differ from those in adults in that most of the former are autosomal-recessively inherited – few have X-linked inheritance – while the latter are often sporadic or autosomal-dominantly inherited. NDDC may be catabolic and/or anabolic conditions, some of which combine maldevelopmental and degenerative features, for instance, peroxisomal biogenesis disorders or congenital disorders of glycosylation. NDDC are often multi...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 8 Neurotrauma
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Colin Smith Traumatic brain injury remains a major cause of morbidity and mortality throughout the world, affecting young and old alike. Pathologic data have been developed through observations of human autopsies and developing animal models to investigate mechanisms, although animal models do not represent the polypathology of human brain injury and there are likely to be significant differences in the anatomic basis of injury and cellular responses between species. Traumatic brain injury can be defined pathologically as either focal or...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 7 Neuropathology of cerebrovascular diseases
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Isidro Ferrer, Noemi Vidal The chapter describes the epidemiology of cerebrovascular diseases, anatomy of the cerebral blood vessels, pathophysiology of ischemia, hypoxia, hypoxemia, anemic hypoxia, histotoxic hypoxia, carbon monoxide damage, hyperoxid brain damage and decompression sickness, and selective cell and regional vulnerability; diseases of the blood vessels including atherosclerosis, hypertensive angiopathy, small vessel disease, inflammatory vascular diseases, cerebral amyloid angiopathies, CADASIL, CARASIL and other disease...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 6 Developmental and perinatal brain diseases
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Homa Adle-Biassette, Jeffery A. Golden, Brian Harding This chapter briefly describes the normal development of the nervous system, the neuropathology and pathophysiology of acquired and secondary disorders affecting the embryo, fetus, and child. They include CNS manifestations of chromosomal change; forebrain patterning defects; disorders of the brain size; cell migration and specification disorders; cerebellum, hindbrain and spinal patterning defects; hydrocephalus; secondary malformations and destructive pathologies; vascular malform...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 5 Cerebrospinal fluid circulation and hydrocephalus
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Ville Leinonen, Ritva Vanninen, Tuomas Rauramaa Hydrocephalus (HC) is classically defined as dynamic imbalance between the production and absorption of cerebrospinal fluid (CSF) leading to enlarged ventricles. Potential causative factors include various brain disorders like tumors causing obstruction of CSF flow within the ventricular system or the subarachnoid space. Classification of HC is based on the site of CSF flow obstruction guiding optimal treatment, with endoscopic third ventriculostomy in intraventricular obstruction and CSF...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 4 Raised intracranial pressure and brain edema
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Ville Leinonen, Ritva Vanninen, Tuomas Rauramaa Acutely increased intracranial pressure (ICP) is a life-threatening neurosurgical emergency. Optimal management strategy is selected according to the causative process. Typical causes are intracranial bleeds like traumatic subdural, epidural, or intracerebral hematoma (ICH); spontaneous ICH, intraventricular hemorrhage, subarachnoid hemorrhage, and hydrocephalus. When occurring without significant brain injury and treated effectively before herniation, a full recovery can be expected. In ...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
Chapter 3 Cellular reactions of the central nervous system
Publication date: 2017 Source:Handbook of Clinical Neurology, Volume 145 Author(s): Gabor G. Kovacs Major cell types of the central nervous system comprise neurons, glial cells (astrocytes, oligodendrocytes, ependymal cells, and microglia), choroid plexus cells, cells related to blood vessels and coverings. These cells show a wide range of reactions to various noxious agents, which can be detected in routine stainings. Some of these reactions are nonspecific to different injuries; however some, such as the appearance of inclusion bodies, can be highly disease-specific. Immunohistochemical markers are widely used in neur...
Source: Handbook of Clinical Neurology - October 4, 2017 Category: Neurology Source Type: research
