Supplementary Material for: Recurrent Acute on Chronic Respiratory Failure in Stiff Person Syndrome

Abstract: Stiff person syndrome (SPS) is an extremely rare disease that presents with episodic painful muscle spasms and progressive muscle rigidity. Recent evidence suggests that SPS can rarely manifest with life threatening respiratory complications. However, the pathophysiology behind respiratory failure in SPS is still not clearly understood. Here we explored an extremely rare case of a 36-year-old African-American female with SPS presenting with multiple episodes of respiratory failure events for the past 9 years. She had an in-situ tracheostomy and was admitted to the hospital for tracheostomy evaluation and decannulation. She initially presented 11 years ago with gait abnormalities, stiffness and spastic episodes. She was diagnosed one year later with SPS after detecting elevated anti-glutamic acid decarboxylase antibody levels in her blood. Through this report we were able to follow a very rare case of SPS that presented with multiple episodes of respiratory failure. We pointed out the importance of early start and regular administration of diazepam, baclofen and IVIg in not only controlling the symptoms and progression of the disease but also in preventing further respiratory failure and possible sudden death.