Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026837 (muscle rigidity)
 1,077 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:[Anesthetic Management for a Patient with Stiff-person Syndrome]. 2701 73