Amyotrophic lateral sclerosis (ALS) is a degenerative disease process that produces both upper and lower motor neuron degeneration. It most commonly affects men 40 to 60 years of age. Frequent initial manifestations include skeletal muscle atrophy, weakness, and fasciculations. With time, atrophy and weakness involve most of the skeletal muscles, including the tongue, pharynx, larynx, and chest. Bulbar involvement includes dysphagia and tongue fasciculations, frequently leading to pulmonary aspiration. ALS has no known treatment, and death is likely within 6 years after the onset of clinical symptoms, usually due to respiratory failure.
General anesthesia in patients with ALS may be associated with exaggerated respiratory depression. ALS patients are vulnerable to hyperkalemia following administration of succinylcholine as a result of lower motor neuron disease. These patients may also show prolonged responses to nondepolarizing muscle relaxants. Bulbar involvement leads to increased risk of pulmonary aspiration. There is no evidence that any specific anesthetic drug or combination of drugs is ideal in these patients. Regional anesthesia is often avoided because of fear of exacerbating disease symptoms. However, epidural anesthesia has been used in ALS patients without neurologic exacerbation or impairment of pulmonary function.
- Stoelting’s Anesthesia and Co-Existing Disease, Chapter 11, pg 262-263
Defined by: CT Bell, MD