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Prolonged Muscle Paralysis Associated Acetylcholinesterase Deficiency

Authors

Nadine Elhage OMS-IV, Elizabeth Gheordunescu D.O, Michael Brennan D.O, Hunter Hayes, D.O. Ahmad Maarouf, M.D. Beaumont Hospital Trenton. Michigan State University College of Osteopathic Medicine.

Introduction:

Acetylcholinesterase Deficiency is a rare autosomal recessive disorder occurring 1 in every 3,200-5,000 people. Usually presents as a persistent paralysis following surgery due to prolonged action of acetylcholine. There have been rare reports in the literature of such a phenomenon.

Case Presentation:

A 70-year-old female was admitted to the hospital with L3 and sacral fractures secondary to a traumatic fall from standing one month prior. She reported non-radiating hip pain after the fall, denied numbness, tingling, bladder, or bowel incontinence. During her hospital stay, she also developed an acute kidney injury and hypokalemia, which resolved prior to surgery with appropriate treatment. The patient was optimized and prepped for surgery that occurred six days after admission. The patient was given succinylcholine 100mg and rocuronium 50mg for intraoperative paralysis. The surgery proceeded uneventfully. Post-operatively, it was noted that the patient did not have adequate twitch response as measured by train-of-four (TOF), indicating that she was still under the effect of paralytics. One dose of Sugammadex was given in the PACU to try and reverse the succinylcholine. The patient did not respond to the reversal agent and was transferred to the intensive care unit for further monitoring while mechanically ventilated. In the ICU she was given an additional dose of Sugammadex, totaling 200 mg, and was again noted to have less than two twitches as measured by TOF stimulation. She was also given a challenge using 5mg of neostigmine out of concern for new-onset MG crisis, possibly exacerbated intraoperatively. It was noted in the intensive care unit that her sedation wore off, so she was given propofol to comfort the patient. Over the next 12 hours, the patient slowly regained muscle function and was able to be extubated with no difficulties. The patient stated that she remembers the events that happened post-operatively and was only paralyzed, which is a new phenomenon not seen in other literature with patients who had prolonged paralysis. Acetylcholine receptor antibodies (AChR)were obtained and found to be less than 0.3 (units), which was in the range of negative, effectively ruling out a potential diagnosis of myasthenia gravis. In consultation with the neurology service, her diagnosis was acetylcholinesterase deficiency that therefore suspected. After the patient regained muscular control, she was discharged later that day with no complications.

Discussion:

While no treatment exists, documentation of acetylcholinesterase deficiency is essential so for future surgeries, patients can remain intubated and mechanically ventilated until they regain movement to be extubated safely. In addition, dibucaine testing to see the level of acetylcholinesterase enzyme deficiency may not be available at all hospitals, ruling out myasthenia gravis by antibody levels and excluding conditions that may cause moderate paralysis should be adequate. For patients with suspected or confirmed acetylcholinesterase deficiency, if receiving muscle paralytic for indicated surgery, the patient should receive an anesthetic to provide adequate sedation to deter the event of awareness under anesthesia that happened in this particular case.

References

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