Emergent Anesthetic Management in a Patient Recently Diagnosed With Stiff Person Syndrome

Stiff person syndrome (SPS) is a rare autoimmune neurological disorder characterized by muscle rigidity and episodic spasms that involve axial and limb musculature. It has important implications during anesthesia, as it leads to gamma-aminobutyric acid (GABA)-mediated inhibitory networks malfunction. This report describes the anesthetic management of a 56-year-old patient with SPS and hereditary spherocytosis undergoing emergent splenectomy due to splenic hematoma and hemoperitoneum after a fall. Total intravenous anesthesia (TIVA) was performed with the adjunctive administration of rocuronium in order to obtain adequate intubation and surgical conditions. Careful management of patients with SPS is strongly suggested given their sensitivity to inhalational anesthetics and neuromuscular blockers, which can lead to hypotonia and muscle weakness requiring maintenance of mechanical ventilation in the postoperative period.