P037: MONITORED ANESTHESIA CARE IN A GI PATIENT WITH STIFF PERSON SYNDROME
Didja Hilmara, MD; Gladys Valerio Reyes, MD; Marie Chantal Policard, MD; Jennifer Hochman Cohn, MD
University of Miami/Jackson Memorial Hospital
Introduction/Background: Stiff person syndrome is a rare autoimmune disease caused by antibodies against glutamic acid decarboxylase, the rate limiting enzyme in GABA synthesis. This results in reduced GABA-mediated inhibitory neurotransmission which causes painful intermittent muscle spasms and muscle rigidity. SPS affects approximately one in one million people, but it is likely significantly underdiagnosed, making its exact prevalence unknown. It is a rare condition that is often misdiagnosed in its early stages, and delayed diagnosis can result in irreversible disability. Among adults, the male-to-female ratio is approximately 1:2. This condition may pose challenges with airway management and choice of anesthetic technique.
Methods: A 67 year old woman with previous medical history of stiff person's disease treated with IVIG, rituximab, and daily prednisone, and history of pulmonary embolism on apixaban presented with hematochezia and suspected upper gastrointestinal bleeding. She was scheduled for EGD under MAC anesthesia. Preoperative anesthesia exam revealed an alert and oriented, wheel-chair bound patient, Mallampati III, small mouth opening, thyromental distance greater than 6cm, midline trachea and full neck ROM, clear and equal bilateral breath sounds and regular rate and rhythm. Neurologic exam revealed mild dysarthria, normal muscle tone, and ability to move all four extremities independently. Home medications included pregabalin, baclofen, atovaquone, modafinil, apixaban, prednisone, and pantoprazole. Monitored anesthesia care was performed with supplemental oxygen via 4L nasal cannula, and the following medications were administered: intermittent propofol boluses totalling 50mg, a 5mg ketamine bolus, and a 100mg lidocaine bolus.
Results: The patient remained hemodynamically stable throughout the case. GI scope was negative for hemorrhage, and the patient’s recovery after anesthesia was uneventful.
Discussion/Conclusion: The effects of monitored anesthesia care on patients with stiff person syndrome have not been well described in the current literature. Existing case reports describe the management of these patients under general anesthesia using volatile anesthetics, neuromuscular blockade, regional anesthetics and TIVA. Since the patient was hemodynamically stable, had an adequate
NPO time, and was not actively experiencing upper gastrointestinal bleeding, we decided that monitored anesthetic care was an appropriate and safe anesthetic technique for this patient. It maintains hemodynamic stability, preserves respiratory function, and eliminates the need for neuromuscular blockade.