Anesthetic management of a parturient with Stiff person syndrome for urgent cesarean delivery

• Axial and limb stiffness and episodic spasms characterize Stiff person syndrome.
• Anesthetic concerns of Stiff person syndrome include prolonged ventilation.
• Both inhaled and intravenous anesthetic agents can produce muscular weakness.
• Epidural anesthesia can be used in parturients with Stiff person syndrome.

Stiff person syndrome is a rare neurologic disorder with an estimated incidence of 1:1 000 000. The underlying pathophysiology is truncal and proximal limb muscle stiffness resulting from continuous co-contracture of agonist and antagonist muscle groups concomitant with superimposed episodic muscle spasms. Loss of gamma-aminobutyric acid-mediated inhibition creates chronic excitation manifested by tonic agonist-antagonist muscle contraction. To date, only three case reports referred indirectly to the anesthetic management of parturients with Stiff person syndrome. The authors describe their management of a parturient with Stiff person syndrome who underwent urgent cesarean delivery under epidural anesthesia.