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[null,null,"Titolo",null,"Emergencies in neuromuscular disorders / / edited by Maxwell Damian and Marianne de Visser"]
[null,null,"Pubbl/distr/stampa",null,"Cham, Switzerland : , : Springer, , [2022]"]
[{"$nulls":4},"©2022"]
[null,null,"ISBN",null,"3-030-91932-3"]
[null,null,"Descrizione fisica",null,"1 online resource (460 pages)"]
[null,null,"Disciplina",null,"616.744"]
[null,null,"Soggetti",null,"Neurological emergencies"]
[null,null,"Lingua di pubblicazione",null,"Inglese"]
[null,null,"Formato",null,"Materiale a stampa"]
[null,null,null,"Livello bibliografico","Monografia"]
[null,null,null,"Nota di bibliografia","Includes bibliographical references and index."]
[null,null,"Nota di contenuto",null,"Intro -- Foreword -- Preface -- Contents -- Contributors -- Chapter 1: Recognition and Assessment of the Neuromuscular Emergency -- Introduction -- Presentation in the Emergency Department -- History-Taking -- Neurological Examination -- Risk Assessment -- Examination of Cardiac, Autonomic, and Respiratory Function and Skin Inspection -- Conclusion -- Self Assessment Questions -- References -- Chapter 2: Diagnostic Tests in the Acute Setting: Strengths and Limitations -- Introduction: Known and Unknown Unknowns -- Laboratory Values -- Electrolytes (Sodium, Potassium, Calcium, Magnesium) -- Serum Creatine Kinase Activity -- Troponins -- Thyroid-Stimulating Hormone (TSH) -- C-Reactive Protein (CRP) -- \"Liver Function Tests': Alanine Aminotransferase (ALT), Aspartate Aminotransferase (AST), and γ-Glutamyl Transferase (γGT) -- Creatinine -- Erythrocyte Sedimentation Rate (ESR) -- Blood Count -- Blood Gas Analysis -- Lactate -- CSF -- Myoglobin -- Immunofixation -- Virology -- Porphyria Screening -- Toxicology Screening -- Autoantibodies -- Soluble Interleukin-2 Receptor -- Ferritin -- Molecular Genetics -- Electrodiagnostic Studies -- Introduction -- Indications -- Caveats -- Brief Summary of Electrodiagnostic Techniques -- Additional Studies Used in a Critical Care Setting -- Phrenic Motor Study -- Diaphragmatic EMG -- Direct Muscle Stimulation (DMS) -- Patterns of Abnormalities Encountered in Neuromuscular Disorders in the Critical Care Unit -- Practical Aspects of Performing Electrodiagnostic Studies in the Intensive Care Setting -- Limitations and Statistical Considerations -- Spirometry -- Imaging -- Imaging in the Detection of Acute Muscle Damage, That Is, "]
[{"$nulls":4},"Oedema -- Imaging for the Detection (and Possibly Classification) of Acute Peripheral Nerve Damage -- Imaging of the Diaphragm -- Imaging of the CNS -- Spinal Imaging -- Brain Imaging."]
[{"$nulls":4},"Stroke-Like Episodes -- White Matter Abnormalities -- Cardiac Imaging -- Histology/Cytology -- Muscle Biopsy -- Nerve Biopsy -- Blood Film Examination -- Self Assessment Questions -- References -- Chapter 3: Respiratory Management in Acute Neuromuscular Disease -- Introduction -- Central Components of Mechanical Respiratory Failure -- Peripheral Component of Mechanical Respiratory Failure -- Measurable Pathophysiology in Mechanical Respiratory Failure -- Clinical Recognition of Acute Respiratory Failure -- Causes of Acute Mechanical Respiratory Failure -- Neurophysiological and Dynamic Testing -- ICU Level of Care -- Tracheostomy -- Liberating the Patient from Mechanical Ventilation -- Summary -- Self Assessment Questions -- References -- Chapter 4: Management of Respiratory Emergencies in Chronic Neuromuscular Disease -- Introduction -- Respiratory Muscle Weakness Due to Neuromuscular Disease -- Insufficient Ventilation -- Bulbar Dysfunction -- Ineffective Cough -- Evaluation -- Respiratory Muscle Weakness -- Diaphragmatic Function -- Cough Assessment -- Symptoms and Signs of Respiratory Failure -- Management of Acute Respiratory Failure -- Mechanical Ventilation -- Interfaces -- NIPPV: Initial Settings and Practical Aspects -- Air Stacking and Mechanical In- and Exsufflation -- Conclusion -- Self Assessment Questions -- References -- Chapter 5: Weaning from the Ventilator and Long-Term Respiratory Support -- Current Concepts on Long-Term Ventilation of Neuromuscular Patients -- Considerations of Respiratory Management in Children -- Patterns of Respiratory Muscle Dysfunction and Spinal and Thoracic Complications in Neuromuscular Diseases -- Setting Up and Follow-Up of Home Ventilation in an Adult with a Neuromuscular Disease -- Initiating Home Ventilation at the Pediatric Age -- Weaning an Adult from Ventilation."]
[{"$nulls":4},"Weaning a Child with Neuromuscular Disease -- Self Assessment Questions -- References -- Chapter 6: Emergencies in Motor Neuron Diseases -- Introduction -- Emergencies in Spinal Muscular Atrophy -- Diagnosis -- Choking -- Respiratory Emergency -- Metabolic Management During Emergency/Surgical Procedures -- Fractures -- Emergencies in Amyotrophic Lateral Sclerosis -- Respiratory Failure -- Choking -- Risks of Gastrostomy Tube Placement -- Laryngospasm and Stridor -- Deep Venous Thrombosis and Pulmonary Embolus -- Concluding Remarks -- Self Assessment Questions -- References -- Chapter 7: Emergencies in Peripheral Neuropathies -- Introduction -- Guillain-Barré Syndrome -- Epidemiology -- Pathophysiology -- Clinical Presentation -- Variants of GBS -- Diagnosis and Diagnostic Investigations -- Differential Diagnosis -- Therapeutic Management -- Initial Evaluation in the Emergency Department -- Management in the Intensive Care Unit -- Immunomodulatory Therapy -- Prognosis -- Other Peripheral Neuropathy Emergencies -- Self Assessment Questions -- References -- Chapter 8: Infectious Diseases of the Peripheral Nerve and Spinal Cord -- Introduction -- Localisation by Site of Involvement -- Cranial Nerves -- Spinal Cord -- Dorsal Root Ganglion, Nerve Roots and Plexus -- Peripheral Nerve -- Subgroups that Warrant Special Attention -- Children, Pregnant Women and Elderly Populations -- Immunosuppression Due to Treatment -- Neuromuscular Emergencies in Low-Income Societies -- The Returning Traveller -- The Non-immunised/Partially Immunised Host -- Specific Infections -- Viruses -- HIV -- Corona Virus: Sars-CoV-2 (COVID-19) -- Rabies -- Bacteria -- Borrelia Burgdorferi (Lyme Disease) -- "]
[{"$nulls":4},"Clostridium Botulinum (Botulism) -- Corynebacterium Diphtheriae (Diphtheria) -- Clostridium Tetani (Tetanus) -- Treponema Pallidum (Syphilis)."]
[{"$nulls":4},"Mycobacterium Tuberculosis (Tuberculosis) -- Helminths -- Schistosomiasis -- Self Assessment Questions -- References -- Chapter 9: The Acute and Emergency Management of Neuromuscular Junction Disorders -- Introduction: The Physiology of Neuromuscular Junction Transmission -- Autoimmune Myasthenia Gravis -- Epidemiology and Clinical Presentation -- Pathophysiology -- Diagnosis of MG -- General Principles of Treatment of MG -- Assessment of the Acutely Unstable MG Patient -- Myasthenic Crisis: Management in the ICU -- Elective ICU Admission in Myasthenia Gravis -- Myasthenia Related to Immune Checkpoint Inhibitors -- Other Neuromuscular Junction Syndromes -- Lambert-Eaton Syndrome -- Botulism -- Nerve Agents -- Congenital Myasthenic Syndromes (CMS) -- Epidemiology and Classification -- Clinical Presentation and Diagnosis -- General Principles of Treatment of CMS -- Pharmacological Treatment of CMS -- Self Assessment Questions -- References -- Chapter 10: Emergencies in Acute Myopathies: Acute Toxic Myopathies, Rhabdomyolysis, and Malignant Hyperthermia -- Rhabdomyolysis and Acute Toxic Myopathies -- Introduction and Definition -- Epidemiology and Clinical Presentation -- Clinical Presentation -- Pathophysiology -- Diagnosis -- Prognosis -- General Principles of Rhabdomyolysis Treatment -- Malignant Hyperthermia -- Introduction and Definition -- Epidemiology -- Clinical Description -- Etiology and Pathophysiology -- Diagnosis -- Principles of Malignant Hyperthermia (MH) Treatment -- Prognosis and Prevention -- Self Assessment Questions -- References -- Chapter 11: Emergencies in Idiopathic Inflammatory Myopathies -- Introduction -- Idiopathic Inflammatory Myopathies -- Epidemiology and Clinical Presentation of Idiopathic Inflammatory Myopathies -- Epidemiology -- Clinical Presentation and Ancillary Investigations -- Muscle Involvement."]
[{"$nulls":4},"Skin Abnormalities -- Respiratory Failure -- Dysphagia -- Systemic Symptoms -- Cardiac Involvement -- Presentation in Children -- Pathophysiology -- Dermatomyositis -- Other Subtypes -- Diagnosis and Classification of Idiopathic Inflammatory Myopathies -- Diagnostic Tests -- Cancer-Related IIM -- General Principles of Treatment for Inflammatory Myopathies -- Emergencies and their Management -- Management of Severe Muscle Weakness -- Management of Severe Dysphagia -- Management of Interstitial Lung Disease -- Management of Cardiac Involvement -- Management of Cancer-Associated Myositis (CAM) -- Management of Immune Checkpoint Inhibitor-Related Myositis -- Management of Severe Cutaneous Lesions -- Conclusion -- Self Assessment Questions -- References -- Chapter 12: The Emergency and Intensive Care Management of Metabolic Myopathies -- Introduction Epidemiology, Pathophysiology, and Clinical Presentation -- Epidemiology and Classification -- Disorders of Glycogen and Glucose Metabolism -- Disorders of Fatty Acid Metabolism -- Pathophysiology -- Disorders of Glycogen and Glucose Metabolism -- Disorders of Fatty Acid Metabolism -- Clinical Presentation -- Disorders of Glycogen and Glucose Metabolism -- Disorders of Fatty Acid Metabolism -- Diagnosis and General Principles of Management -- Diagnostic Testing in Glycogenoses -- Diagnostic Testing in Disorders of Fatty Acid Metabolism -- General Principles of Management -- Management of Emergencies in Metabolic Myopathies -- Glycogen Storage Disorders -- Fatty Acid Oxidation Disorders -- Planned Anaesthesia and Ventilation in ICU -- Self Assessment Questions -- References -- Chapter 13: Intensive Care "]
[{"$nulls":4},"Implications in Primary Mitochondrial Disease -- Introduction -- Diagnostic Work-Up in Suspected Mitochondrial Disease at the ICU -- Genetics of Mitochondrial Disease."]
[{"$nulls":4},"General Approach to Critical Illness in Patients with Mitochondrial Disease."]