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Patients with acute life-threatening neuromuscular disease require close cooperation between neuromuscular and intensive care specialists to achieve the best possible outcomes. The problems encountered by these patients are different from those in traditional neuromuscular practice, and neurologists consulting in the ICU need a specific skill set to provide useful guidance. However, outcomes can be very good if treatment is instituted effectively. This review aims to provide an overview of the most important neuromuscular conditions encountered in the ICU and enable a practical approach to patient management.New research has provided improved knowledge of the impact of acute neuromuscular failure on the mechanics of respiration, on the categories of neuromuscular disease in the ICU, and on the main factors influencing outcomes. Pitfalls and risks in ICU treatment are better understood.Evidence-based algorithms for monitoring and treatment have been developed. These advances enhance the role of the neuromuscular specialist in acute care. The principles of best practice are discussed in this review.