Intranasal midazolam for treating acute respiratory crises in a woman with stiff person syndrome

Abstract

Stiff person syndrome (SPS) is a rare neurologic disorder characterized by progressively worsening rigidity and spasms of the axial and limb muscles. Dyspnea has been recently recognized as a common symptom in SPS,1 and life-threatening respiratory crises have been occasionally reported and suspected to be responsible for sudden death in these patients.2,3 The pathophysiologic mechanisms of these respiratory manifestations remain unclear. Some authors have hypothesized that rigidity and/or spasm of the muscles of the trunk could prevent normal rib cage movements and excursion of the diaphragm.1