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.

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).