Neuraxial anesthesia for Kennedy’s motor neuron disease. A case report.
Clinical Case Reports
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Introduction: Anesthetic management of patients with motor neuron disease represents a challenge. Kennedy’s disease (KD), also known as spinal and bulbar muscle atrophy (SBMA), is a rare, adult-onset, X-linked motor neuron disease characterized by progressive spinal and bulbar motor neuron disease and muscle atrophy. In this report, we describe a case of successful application of neuro-axial anesthetic (NA) during hip arthroplasty in KD through a combined spinal-epidural anesthetic.
Materials and Methods: Informed consent was obtained from the patient for publication. As the case report is devoid of patient identifiable information, it is exempt from IRB review requirements as per Virginia Commonwealth University Healthcare policy. The authors were directly responsible for pre-operative evaluation, administration of neuro-axial anesthesia and monitored sedation during the surgical procedure. Daily post-operative follow-up and management of the epidural catheter were conducted by the anesthesia team per institutional protocols. The patient’s medical record was reviewed to depict outcome based on outpatient clinic evaluations.
Case Report: We are reporting a 60-year-old gentleman with KD who presented with traumatic displaced intertrochanteric fracture of left femur. The patient’s neurological condition was advanced with pronounced dysarthria, dysphagia, and generalized weakness. The patient had a history of failure to extubate after general endotracheal anesthesia (GETA) requiring prolonged mechanical ventilation and tracheostomy that was later reversed. He was evaluated by anesthesiology and deemed a suitable candidate for NA. The patient tolerated the anesthetic and surgical procedures well and was discharged on postoperative day 3 after an uneventful recovery.
Discussion: The disease phenotype is a function of mutated androgen receptor gene resulting in the accumulation of defective protein which leads to motor neuron loss and myopathy. Indeed, previous reports indicate a high risk of perioperative complications associated with GETA. Muscle relaxation poses the risk of hyperkalemic crises with succinylcholine or extended neuromuscular blockade with non-depolarizing agents leading to failure to extubate post-operatively and prolonged ventilation. Volatile anesthetic agents have also been implicated in severe rhabdomyolysis and anesthesia-related cardiac arrest in patients with Becker’s muscle dystrophy. The bulbar dysfunction characteristic of KD also inherently pose a heightened risk of aspiration and/or laryngospasm. Rapid sequence intubation (RSI), preferably without muscle relaxation and total intravenous anesthesia (TIVA) is, therefore, the preferred GETA technique. Neuro-axial anesthesia, when indicated, can spare the patient the risks associated with GETA, however, patients should be counseled on relatively higher morbidity of complications inherent to NA like high spinal and potential for transient neurological symptoms (TNS).
Conclusion: Combined spinal-epidural anesthesia and monitored sedation can be safely and effectively deployed in selected cases of KD.
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