Botulism

Botulism (Orphanet code 1267) is a rare but potentially life-threatening neuroparalytic illness caused by toxins produced by the bacterium Clostridium botulinum, and occasionally by toxin-producing strains of Clostridium butyricum and Clostridium baratii. The botulinum neurotoxin blocks acetylcholine release at the neuromuscular junction, leading to flaccid paralysis. The disease primarily affects the nervous system and skeletal muscles. There are several forms: foodborne botulism (caused by ingestion of preformed toxin in contaminated food, ICD-10 A05.1), infant botulism (caused by intestinal colonization and in vivo toxin production in infants), wound botulism (from toxin produced in infected wounds), and iatrogenic botulism. Adult intestinal colonization botulism is a rare variant similar to the infant form. Key clinical features include symmetric descending flaccid paralysis, typically beginning with cranial nerve involvement causing diplopia (double vision), blurred vision, ptosis (drooping eyelids), dysarthria (slurred speech), dysphagia (difficulty swallowing), and dry mouth. The paralysis can progress to involve the respiratory muscles, potentially leading to respiratory failure requiring mechanical ventilation. Gastrointestinal symptoms such as nausea, vomiting, abdominal cramps, and constipation may precede or accompany neurological symptoms, particularly in foodborne botulism. Importantly, patients remain alert and afebrile, and sensation is preserved. Treatment depends on the form of botulism. For foodborne and wound botulism in adults, equine-derived heptavalent botulinum antitoxin is administered as early as possible to neutralize circulating toxin and prevent disease progression, though it cannot reverse existing paralysis. For infant botulism, human-derived botulism immune globulin (BabyBIG) is the standard treatment. Supportive care, including intensive care monitoring and mechanical ventilation when needed, is critical. Wound botulism also requires surgical debridement and antibiotics. With modern intensive care, mortality has decreased significantly, though recovery can take weeks to months as new nerve terminals must regenerate.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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