Neuromuscular emergencies may be defined as disorders or exacerbations of diseases of the peripheral nervous system that are rapidly progressive and potentially life-threatening. Such disorders can affect any level of the peripheral nervous system, from the muscle to the anterior horn cell. While their clinical manifestations may vary, severe morbidity and mortality are most frequently the result of neuromuscular respiratory failure. Acute, generalized weakness may result from multiple localizations within the peripheral nervous system. Features such as the presence or absence of sensory symptoms, reflex changes, distribution of muscle weakness, extraocular muscle involvement, and the involvement of other organ systems can aid the clinician in localizing the patient’s disorder. Medical history collection, prompt clinical evaluation, blood tests, electromyography and electroneurography, and cerebrospinal fluid (CSF) can all help to perform prompt diagnosis.

The epidemiology of acute neuromuscular disorders is difficult to evaluate, differing significantly in countries and varying significantly among acute emergency settings. The sudden appearance of symptoms and signs can constitute the first presentation of disease or a possible complication of an already diagnosed neuromuscular disorder (NMD), which can remain stable for years with sudden worsening. Among the most commonly observed NMDs at emergency departments are Guillain Barrè syndrome and its variants, rare life-threatening polyneuropathies with an estimated reported mortality rate of between 3% and 13%. Neuromuscular genetic disorders such as channelopathies, for example, hypokalemic periodic paralysis, can present with the sudden onset of generalized or focal flaccid paralysis associated with hypokalemia. Myasthenic crisis is a complication of myasthenia gravis characterized by a worsening of muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation; current mortality is still 4% and primarily the result of comorbid medical conditions. Even metabolic disorders, such as porphyria, can present with neurological symptoms, such as acute neuromuscular symptoms and signs. Botulism, a rare, neurotoxin-mediated life-threatening disease, typically produces a distinctive syndrome of cranial nerve palsies that can be followed by bilateral, symmetric, descending flaccid paralysis, affecting the proximal before the distal limb musculature, that might progress to respiratory failure and death.

The aim of this Special Issue is to improve the prompt recognition, diagnosis, and management of neuromuscular diseases in emergency departments, for colleagues working in emergency wards and intensive care units (ICU), including infectious motor neuronopathies, inflammatory polyradiculopathies, and disorders of neuromuscular transmission. New challenges can be considered the prompt recognition and diagnosis, and the institution of specific therapies or supportive care to prevent mortality and mitigate morbidity.

Potential topics include but are not limited to the following:

• Diagnosis, evaluation, management, and differential diagnosis of Guillain Barrè syndrome
• Chronic inflammatory demyelinating polyneuropathy and atypical variants
• Prompt recognition and treatment of channelopathies
• Diagnosis and management of myasthenia gravis, with special focus of myastenic crisis
• Potential development of amyotrophic lateral sclerosis into an emergency disorder and related treatment and ethical concerns
• Main causes of rhabdomyolysis
• Management of glycogen and lipid storage diseases and mitochondrial disorders, such as myoclonic epilepsy with ragged red fibers (MERRF) and mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)
• Recognition and treatment of botulism
• Recognition of manifestations and treatment of tetanus
• Development of Duchenne muscular dystrophy into an emergency disease
• Development of Steinert myotonic dystrophy into an emergency neuromuscular disorder
• Recognition and treatment of polymyositis and dermatomyositis
• Diagnosis and management of spinal muscular atrophy and possible emergencies in young patients
• Proposed algorithms for the management of acute neuromuscular disorders and their adaptation to local settings
• Exploration of management of neuromuscular disorders with multidisciplinary approaches using ICU and emergency doctors
• Prompt recognition and treatment of respiratory involvement in neuromuscular disorders