Decubitus ulcers are rare. The primary cause of death among patients electing to use ventilatory support is pulmonary infection. Cause is unknown. Often, neurologists need several weeks to rule out all other possible diagnoses in a patient presenting with upper and lower motor neuron signs. Clinically, the diagnosis is made with the support of electromyography and by exclusion of other neurologic diseases.
Electromyography shows fibrillation and fasciculations.
The motor units are polyphasic and have high amplitude and long duration. When diagnosis is unclear, perform CT scans and contrast myelography to visualize the cervical spine. Be familiar with ALS and entertain the diagnosis in patients presenting with upper and lower motor signs. Manage symptoms and ensure that emotional support is available to patients and their families. Denial is common in the early stages of the disease.