Systemic Botulinum Toxicity in an Emery-Dreifuss Muscular Dystrophy Patient Secondary to a Botox Injection (P4.022)

Objective: To present a unique case of a rapid onset systemic botulinum toxicity in a patient with Emery-Dreifuss muscular dystrophy after receiving an intrapylorus Botox injection. Background: Emery-Dreifuss muscular dystrophy is an inherited disorder. It is characterized by progressive muscle weakness and cardiac involvement1. Gastrointestinal disturbances such as chronic dysmotility can occur in such syndromes2. Endoscopic Intrapyloric Botox injections have been used a treatment for patients with severe gastroparesis. However, limited data is available with patients who have an underlying muscular dystrophy who have received botulinum toxin injection for a dysmotility syndrome. Design/Methods: A 27-year-old female with Emery-Dreifuss muscular dystrophy with a past medical history of chronic dysmotility. She had tried several medications for dysmotility, however she continued to experience severe regurgitation. She received a 100 units of Botox injection into the pylorus. Two hours later she presented to the Emergency Department with progressive weakness, headache and dyspnea. Physical exam included notable decreased strength of her neck muscles, upper and lower extremities. She also had decreased breath sounds on pulmonary auscultation. Deep Tendon Reflexes were absent throughout, and she was unable to ambulate. Results: Initial labs including a CBC, BMP, UA were negative. CT head was negative. A lumber puncture was completed as well, showing no albuminocytologic dissociation. Since her weakness and dyspnea worsened, she was transferred to the Intensive Care Unit for further monitoring. Her respiratory status began to decline with decreasing negative inspiratory force. Conclusions: Centers for Disease Control (CDC) was called and Heptavalent Botulism Anti-toxin was administered within 24 hours. Her respiratory status improved and had improving measurable Negative Inspiratory Forces. The patient also increased her strength and dyspnea resolved over the course the next few days. Eventually she was transferred out of the Intensive Care Unit. She had improved back to her baseline function, was able to ambulate and was discharged. Disclosure: Dr. Kaul has nothing to disclose. Dr. Waseem has nothing to disclose. Dr. Thomas has nothing to disclose. Dr. Stommel has nothing to disclose. Dr. Feldman has nothing to disclose. Dr. Lee has nothing to disclose. Dr. Cohen has nothing to disclose.