ICU · Neurocritical care / neuromuscular

Motor Neuron Disease & Neuromuscular Weakness in ICU — ALS, NIV & End-of-Life

Also known as Motor neuron disease · MND · Amyotrophic lateral sclerosis · ALS · Neuromuscular respiratory failure · Riluzole · Non-invasive ventilation · El Escorial criteria · Fasciculation · Critical illness polyneuromyopathy · Multifocal motor neuropathy · Bulbar weakness

Motor neuron disease (MND), or the amyotrophic lateral sclerosis (ALS), is a progressive degeneration of the upper and the lower motor neurons, causing a mixed UMN and LMN signs (the muscle wasting, the fasciculations, and the brisk reflexes) in the SAME limb, with the sensation and the sphincters SPARED. It is relentlessly progressive and fatal — the typical terminal event is the respiratory failure from the diaphragm weakness. In the ICU, the MND presents with a type 2 respiratory failure or an aspiration from the bulbar weakness. The management: the riluzole (extends the survival by about 3 months); the non-invasive ventilation (the standard for the symptomatic respiratory weakness — the FVC under 50 per cent or the orthopnoea; improves the survival and the quality of life); the PEG nutrition before the weight loss; and the advance care planning (the decision to ventilate is ethically complex). The neuromuscular weakness in the ICU has a broad differential — the GBS, the myasthenia, the MND, the critical-illness polyneuromyopathy, the electrolyte disorders, and the cord lesion.