title: [Anesthetic Management for a Patient with Stiff-person Syndrome].
abstract: The stiff-person syndrome (SPS) is a rare autoimmune neurologic disorder that affects the gamma-aminobutyric acid (GABA) mediated inhibitory network in the central nervous system with anti-glutamic acid decarboxylase (GAD) antibodies. SPS is characterized by muscle rigidity and painful episodic spasms in axial and lower limb muscles. This case report describes successful peri-operative management of a 61-year-old female (height, 158 cm; weight, 60 kg, ASA-PS 2) with her right upper arm fracture who was scheduled for open reduction and internal fixation. This patient had bulbar paralysis, dysphagia and muscle rigidity associated with a high titer of anti-GAD auto antibodies (2,800 U x ml(-1)). She was diagnosed as SPS and has been treated with predonisolone (30 mg x day(-1)) and diazepam (20 mg x day(-1)) for 1 year. Predonisolone (15 mg) and diazepam (30 mg) was given orally before induction of general anesthesia with propofol, remifentanil and rocuronium bromide. Posture change from supine to beach-chair position led to sudden drop in blood pressure to 38/25 mmHg, which recovered promptly by injecting intravenous ephedrine hydrochloride (28 mg) and hydrocortisone (100 mg). Postanesthetic course was uneventful without postoperative neurologic abnormalities.
authors.forename: Kumiko
authors.initials: K
authors.lastname: Nakamura
authors.forename: Kohei
authors.initials: K
authors.lastname: Murao
authors.forename: Michiyo
authors.initials: M
authors.lastname: Kimoto-Shirakawa
authors.forename: Kazuyo
authors.initials: K
authors.lastname: Takahira
authors.forename: Farah
authors.initials: F
authors.lastname: Toorabally
authors.forename: Koh
authors.initials: K
authors.lastname: Shingu
mesh_terms: D000758:Anesthesia / Q000379:methods*; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology; D006801:Humans; D008875:Middle Aged; D011187:Posture; D016750:Stiff-Person Syndrome / Q000503:physiopathology*
chemical_list: D005968:Glutamate Decarboxylase