A series is reported of five patients with acute respiratory failure complicating neuromuscular diseases, three of whom required assisted ventilation. Initially the arterial carbon dioxide tension fell to levels below the normal range in all the patients and this change reflects alveolar hyperventilation which is probably produced by a combination of fear, small airway collapse, and reflex tach...

Children with neuromuscular diseases present a wide range of clinical manifestations and clinical implications for the anaesthesiologist. Neuromuscular diseases in children affect muscle strength by either directly weakening the muscle fibrils or indirectly by a degenerative nerve supply and weak neuromuscular junction. Of the more than 200 neuromuscular disorders known, the vast majority are g...

UNLABELLED Neuromuscular diseases represent an important group in pediatric pathology. Immunohistochemistry together with clinical examination and morphologic exam are very important in the diagnosis of neuromuscular disorders. PATIENTS AND METHODS One hundred children diagnosed with neuromuscular disorders were included in a prospective-retrospective study in 25 years. RESULTS There were 5...

New therapies developed over the past 3 years for previously intractable diseases of skeletal muscle, neuromuscular junctions, peripheral nerves, and motor neurons are now being incorporated into our standard neuromuscular clinical practice. The past 3 years were also marked by important advances in our understanding of the pathogenesis and pathophysiology of inherited and acquired neuromuscula...

Metabolic myopathies are characterized by the dysfunction of several metabolic pathways that results in a deficiency fuels required to generate energy for muscle contractions [...]