Decubitus ulcers are rare. The primary cause of death among patients electing to use ventilatory support is pulmonary infection. The specific cause is unknown. If a history of slow progression of lower motor neuron disease is lacking, consider disorders such as myasthenia gravis and Guillain-Barr syndrome. Often, neurologists need several weeks to exclude all other possible diagnoses in a patient presenting with upper and lower motor neuron signs.
Electromyography shows fibrillation and fasciculations.
The motor units are polyphasic and have high amplitude and long duration. Emergency physicians should be familiar with this disease and should consider the diagnosis in patients with only motor syndromes. Patients initially are cared for symptomatically, and emotional support should be available to the patient and family. Discussion of a new diagnosis of ALS in the ED is inappropriate before further evaluation can occur. If a living will or a declaration for a natural death is in place, keep patients comfortable and do not intubate.