Anesthesia in a patient with Stiff Person Syndrome

Abstract

Stiff Person Syndrome (SPS), typified by rigidity in muscles of the torso and extremities and painful episodic spasms, is a rare autoimmune-based neurological disease. Here we present the successful endotracheal intubation and application of TIVA without muscle relaxants on an SPS patient. A 46 years old male patient was operated with ASA-II physical status because of lumber vertebral compression fracture. After induction of anesthesia using lidocaine, propofol and remifentanil tracheal intubation was completed easily without neuromuscular blockage. Anesthesia was maintained with propofol, remifentanil and O2/air mixture. After a problem-free intraoperative period the patient was extubated and seven days later was discharged walking with aid. Though the mechanism is not clear neuromuscular blockers and volatile anesthetics may cause prolonged hypotonia in patients with SPS. We think the TIVA technique, a general anesthetic practice which does not require neuromuscular blockage, is suitable for these patients.

Anesthetic management of a patient with Stiff-Person Syndrome and endometrial cancer for robotic surgery: A case report

“…[10][11][12] since then, many more reports have demonstrated safe use of various anesthetic agents and techniques in SPS patients. Use of volatile anesthetics, muscle relaxants, regional anesthetics, and total intravenous anesthesia (TIVA) with propofol have all been described in the literature for SPS patients without complication [13][14][15][16][17][18]. Our patient received sevoflurane, rocuronium, and sugammadex along with judicious amounts of benzodiazepine, opioid and dexmedetomidine without postoperative complications.…”

Remimazolam and Remifentanil Anesthetics for an Adolescent Patient with Stiff-Person Syndrome: A Case Report

“…The estimated prevalence of SPS is 1 of 1000,000 2 ; therefore, there are few reports on the general anesthetic management of patients with SPS. 3–5 The pathophysiology of SPS involves GAD, an enzyme essential for GABA synthesis. 6 Furthermore, SPS has been reported to be caused by atrophic Renshaw cells.…”

“…It has been reported that prolonged weakness after general anesthesia may be a result of enhanced central GABAergic transmission due to the interaction between the volatile agent and commonly used medication for SPS, such as baclofen. 4 Additionally, a patient who was anesthetized with total intravenous anesthesia, including propofol showed postoperative hypotonia, despite recovery from muscle relaxant on train-of-four monitoring. 8 Remimazolam has several advantages in patients with SPS.…”

Successful Anesthetic Management of Patient with Stiff Person Syndrome

“…Due to extremely low incidence of the disease, limited data is available on the anesthetic management of patient with SPS. 6 We used spinal anesthesia (bupivacaine and fentanyl) for total hip replacement because general anesthesia might have been associated with delayed emergence and recovery. This technique provides suitable condition for the surgery.…”