Abstract

Objective: Describe the epidemiological, neurological and electrodiagnostic features of 12 prisoners with food borne botulism from self-made alcohol. Background Botulism causes rapidly progressive weakness with prominent dysphagia, ophthalmoparesis, and autonomic dysfunction including pupillary abnormalities. Nerve conduction studies (NCS) typically reveal reduced compound muscle action potential (CMAP) amplitudes with an incremental response to rapid (30-50 hertz) repetitive nerve stimulation (RNS) in 50%. Design/Methods: Twelve adult prisoners developed confirmed botulism after consuming alcohol made from fresh and canned fruit and potatoes (Pruno). All patients were triaged in the emergency department or via teleconference in the prison infirmary; 8 were admitted. Clinical and electrodiagnostic features are reported on the admitted patients. Each underwent NCS including 3 and 50 hertz RNS of the median and ulnar nerves. Results: Serial neurologic examinations revealed bulbar weakness with a notable lack of pupillary abnormalities. Three patients had rapidly progressive respiratory failure requiring intubation, and two developed descending proximal predominant weakness. Determination of the C. Botulinum subtype is pending at the time of submission. NCS were normal in 5 patients. The three most severely affected had mildly reduced compound muscle action potential amplitudes. No patient had CMAP facilitation following exercise, and only the weakest had a 30% increment following 50 hertz RNS. Conclusions: We present features of an outbreak of Pruno associated botulism. Although pupillary findings are commonly cited in the literature as a core feature of botulism, they were uniformly absent. The classic electrodiagnostic features of reduced CMAP amplitudes and incremental response to rapid RNS were also absent in all but 1 patient. These findings emphasize the fact that botulism remains a clinical diagnosis. Absence of pupillary and NCS abnormalities do not exclude botulism and patients with a suspicious exposure history and clinical examination should undergo serum testing and appropriate therapy. Supported by: R01DK064814 (AGS, JRS). Disclosure: Dr. Chung has nothing to disclose. Dr. Burbank has nothing to disclose. Dr. Afra has nothing to disclose. Dr. Hoesch has nothing to disclose. Dr. Singleton has received personal compensation for activities with Medical Review Institute. Dr. Leydard has nothing to disclose. Dr. Dolan has nothing to disclose. Dr. Smith has received personal compensation for activities with Allergan, Baxter Bioscience, Merz, Pfizer and NeurogesX as a consultant. Dr. Smith has received personal compensation in an editorial capacity for serves as the Associate Editor for Education at AAN.com. Dr. Smith has nothing to disclose.

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