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This comprehensive book addresses the acute emergencies in neuromuscular disease from a novel perspective, focusing on clinical management and treatment of these disorders rather than the more traditional neuropathology, neurogenetics, and neurophysiology approaches. The book fills the gap in guidelines by providing an evidence-based guidance for the clinical adult or pediatric neurologist confronted by an acutely ill patient in a potentially life-threatening situation.
The book is structured according to pathophysiological principles, but each chapter is strictly organised around case vignettes that emphasize clinical relevance allowing the non-specialist to access complex scientific backgrounds. The electronic supplementary material and multimedia are provided as an integral tool to facilitate accessibility and understanding of a notoriously difficult field of neurology. Finally, the role of patients, who are increasingly involved in many management decisions, is highlighted in a chapter on ethical issues discussing withholding or withdrawing life-sustaining therapy, patient end-of-life values, wishes and preferences, and on advanced directives.
The publication is aimed equally at the non-specialist neurologist in frontline clinical practice confronted by a rare disorder needing immediate management decisions, at the clinical specialist referring for guidance in specific management aspects, and at the specialist researcher keen to maintain clinic relevance.
Maxwell Damian trained in Germany and the UK, and specialises in genetic muscle disease and in neurocritical care. He was consultant and director of the neurological ICU in the universities of Giessen and Dresden, Germany, before continuing work in the UK, specialising in critical care aspects of neuromuscular disease and in research on hypoxic brain injury. He has been co-chair of the Neurocritical Care scientific panel of the European Academy of Neurology, 2015-2020; chair of the specialist group in Neurocritical Care of the Association of British Neurologists; chair of the accreditation committee of the Neurocritical Care Society, USA. He retired from Cambridge University Hospitals in 2020 but continues research work at the University Department of Anaesthetics, Cambridge University and at the Essex Cardiothoracic Centre, UK.
Marianne de Visser is an adult neurologist at the Amsterdam University Medical Centers, location AcademicMedical Center in Amsterdam, The Netherlands and emeritus Professor of Neuromuscular Diseases. She was trained at the University Hospital of Amsterdam. In 1988 she was a visiting scientist at Dr. Andrew Engel's lab at Mayo Clinic, Rochester, Minnesota where she performed ultrastructural studies on skeletal muscle in dermatomyositis. She obtained a position at the Department of Neurology of the Academic Medical Center in Amsterdam and was appointed as Professor of Neuromuscular Diseases at the University of Amsterdam in 1993.
Her research interests are rare neuromuscular disorders such as myositis, hereditary neuropathies, postpolio syndrome and motor neuron disorders. Her work on ALS kindled interest in palliative care.
Diagnostic assessment in acute neuromuscular disease.- The differential diagnosis of the patient with acute neuromuscular disease and acute flaccid paralysis.- The clinical assessment of the neuromuscular emergency.- Clinical assessment in the child with an acute neuromuscular illness.- Antibody testing and immunological assessment in acute neuromuscular disorders.- Testing of the patient with exercise intolerance and acute rhabdomyolysis.- Electrophysiological assessment of acute neuromuscular disorders and EMG in the ICU.- Assessment of autonomic function.- Nerve and muscle biopsies in the acutely ill patient and histological assessment.- Imaging of the acutely weak patient.- Molecular and genetic testing algorithms in the acutely ill neuromuscular patient.- The neurology of acutely failing respiratory mechanics and its assessment.- Categories of acute neuromuscular disorders.- Poliomyelitis and acute infectious disorders of the motor neurone.- The emergency presentation of Amyotrophic lateral sclerosis and atypical motor neurone disorders (text; tables/flow charts).- Acute presentation of inherited and metabolic neuropathies.- Acute inflammatory neuropathy - Guillain Barre syndrome and its differential diagnoses.- Myasthenia Gravis and myasthenic emergencies.- Botulism, and the Lambert-Eaton myasthenic syndrome.- Congenital myasthenic syndromes and their emergency presentation- Channelopathies and disorders of skeletal muscle membrane excitability.- Rhabdomyolysis and myoglobinuria.- Acute emergencies in childhood muscular dystrophies.- Emergencies in advanced muscular dystrophy.- Acute cardiac failure, cardiomyopathy, and cardiac dysrhythmia in muscle disease.- Congenital myopathies and acute emergencies.- Metabolic myopathies and their acute presentation.- Mitochondrial myopathies: acute presentation and emergency management.- Endocrine and toxic myopathies.- Acute inflammatory and paraneoplastic myopathies.- Infectious and granulomatous myopathies.- C. Management problems in acute and emergency neuromuscular disorders.- 1. ICU-acquired weakness and neuromuscular complications of critical care- 2. Weaning from the ventilator and liberation from tracheotomy in the neuromuscular patient .- 3. Palliative care and advanced decisions in neuromuscular disease.- 4. Acute neuromuscular disorders in challenging environments.- D. ESM (media links; algorithms; self-assessment tools; MCQs).
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