Abstract
Acute neuromuscular diseases may need intensive care unit (ICU)-level care. Myasthenia gravis is the prototypical autoimmune disorder involving the neuromuscular junction, while Guillain-Barré syndrome (or acute inflammatory demyelinating polyneuropathy) is the most common etiology of flaccid paralysis in the United States, and both can lead to respiratory failure necessitating invasive ventilatory support. More specifically, neuromuscular disorders may affect the respiratory system directly by impairing mechanics or indirectly by oropharyngeal dysfunction causing inability to clear secretions. Important bedside measures to assess the strength of respiratory muscles include forced vital capacity (FVC), maximal inspiratory pressure (MIP) (also known as negative inspiratory force (NIF)), and maximal expiratory pressure (MEP). A combination of serologic, cerebrospinal fluid (CSF), and electrophysiologic testing is used to diagnose patients with neuromuscular disease. Critical illness polyneuropathy and critical illness myopathy may develop with prolonged stays in the ICU. Plasmapheresis and intravenous immunoglobulin (IVIG) remain the cornerstones of acute treatments in MG and GBS. Despite advances in care, unfortunately, recovery may be slow or incomplete in these patients.
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Daneshmand, A., Wijdicks, E.F.M. (2020). The Care of Patients with Neuromuscular Disease in the Neurocritical Care Unit. In: Nelson, S., Nyquist, P. (eds) Neurointensive Care Unit. Current Clinical Neurology. Humana, Cham. https://doi.org/10.1007/978-3-030-36548-6_18
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