Anesthetic Considerations of Stiff-Person Syndrome: A Case Report.

Stiff-person syndrome (SPS) is a neurologic disorder characterized by painful involuntary episodes of severe muscle rigidity affecting the axial muscles and extremities. Although the etiology of SPS is unknown, it is suspected to involve the synthesis of γ-aminobutyric acid (GABA). Symptoms of SPS are precipitated by sudden unexpected movements, noises, and stress. Additionally, SPS has been linked with various autoimmune disorders, including diabetes mellitus, thyroid disease, pernicious anemia, and certain cancers. Because of the effect of SPS and SPS medications, inhalational agents and neuromuscular blockers have the potential to cause prolonged hypotonia following anesthesia, resulting in respiratory failure despite full reversal of neuromuscular blockade. In documented case reports, the outcomes of using general anesthesia with inhalational agents and neuromuscular blockers in patients with SPS varied. This case report highlights the anesthetic management of a 56-year-old woman with diagnosed SPS undergoing a hemicolectomy for a colon mass using total intravenous anesthesia.

Stiff-man syndrome: a rare disorder of the central nervous system.

Stiff-man syndrome is a rare disorder of the central nervous system characterized by painful involuntary stiffening of axial muscles accompanied by spasms. Symptomatology is often precipitated by tactile or emotional stimuli, volitional movements and startling noises. It is occasionally associated with epilepsy and endocrine disorders, including insulin-dependent diabetes mellitus. Certain diagnostic criteria are essential for the diagnosis of Stiff-man syndrome since the initial clinical manifestations are similar to those of other neuromuscular diseases. Research findings support the theory of central nervous system autoimmunity with resultant impairment of neuronal pathways as the probable pathogenesis of Stiff-man syndrome.