Background:
Amyotrophic lateral sclerosis is a progressive neurodegenerative disease primarily affecting the upper and lower motor neurons. Patients present with a variety of clinical manifestations inevitably resulting in death secondary to respiratory insufficiency from muscle weakness and consequential pulmonary complications. Despite the lack of universal consensus on the ideal anesthetic approach to amyotrophic lateral sclerosis patients undergoing lower extremity total joint surgery, there are few noteworthy anesthetic considerations in this cohort.
Case report:
A 75-year-old male with multiple medical comorbidities, including a recent diagnosis of amyotrophic lateral sclerosis, presented to a large academic medical center for a right total hip arthroplasty revision. The patient's preoperative neurologic examination demonstrated brisk deep tendon reflexes, visible fasciculations of lower extremities, and motor weakness of upper and lower extremities. Serology demonstrated an elevated creatine kinase, and an electromyography study showed active denervation in the cervical, thoracic, and lumbosacral regions. After a careful risk-benefit analysis was performed, involving a multidisciplinary team approach, the patient successfully underwent the surgical procedure with a spinal anesthetic and minimal sedation. Perioperative course was unremarkable, and there were no neurologic complications in the first 6 months after surgery.
Conclusion:
Patients with amyotrophic lateral sclerosis present unique challenges for anesthesia providers. General anesthesia may potentially worsen respiratory function; therefore, alternative methods to avoiding airway manipulation should be considered. Conversely, regional techniques may carry the risk of exacerbating pre-existing neurologic symptoms. Currently, no definite guidelines exist on the perioperative anesthetic management of amyotrophic lateral sclerosis patients; ultimately, the decision to perform regional anesthesia should be based on analyzing a patient's risk against the potential benefits.