Main description:

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.

Contents:

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).