Table of Contents
Definition / general | Epidemiology | Etiology | Clinical features | Diagnosis | Prognostic factors | Case reports | Treatment | Diagrams / tables | Microscopic (histologic) images | Electron microscopy images | Differential diagnosis | Additional referencesCite this page: Weisenberg E. Clostridium botulinum. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/coloncbotulinum.html. Accessed April 18th, 2024.
Definition / general
- Botulism is a disease caused by botulinum toxin ("botox"), a potent neurotoxin produced by Clostridium botulinum or closely related microorganisms such as C. baratii or C. butyricum
Epidemiology
- Botulism is uncommon in the developed world
- According to the CDC in the United States, about 145 cases are reported each year, 65% in infants, 20% wound related and 15% foodborne
- Most cases are associated with eating raw or undercooked food containing botulinum toxin or C. botulinum spores
- Clusters of disease associated with eating the same food are common
- Canned (especially home canned alkaline foods), vacuum packed food and fermented or smoked foods are often implicated
- Infant botulism is related to ingestion of spores which colonize and release toxin in the intestine; may be related to honey ingestion (MMWR 2003;52:21)
- Wound botulism is caused by toxin released by C. botulinum that has contaminated a wound; may follow heroin injection (Euro Surveill 2013;18:20630, Anaerobe 2014;30:108)
- Rare cases are associated with inhalation of spores, sometimes in a health care setting
- Recently, iatrogenic botulism has been reported due to botulinum toxin for cosmetic or therapeutic purposes (Clin Neuropharmacol 2012;35:254, Clin Neuropharmacol 2010;33:158, Clin Neuropharmacol 2007;30:310, JAMA 2006;296:2476)
Etiology
- Clostridium are toxin producing, spore forming, anaerobic, gram positive bacilli found in soil and marine sediment
- Botulinum toxin is one of the most dangerous substances known; one millionth of a gram is a lethal dose
- It is a potent neurotoxin that blocks the presynaptic release of acetylcholine across the neuromuscular junction, leading to a toxic neuropathy
- Eight toxins have been identified, A-H; A, B, E, and F have been associated with human disease
- Foodborne botulism is associated with ingesting food containing botulinum toxin while infant botulism is associated with ingesting spores that colonize the intestines and produce toxin; disease in adults via a similar mechanism is rare, but may occur (adult intestinal colonization botulism)
- Wound, inhalation and iatrogenic botulism are discussed above under Epidemiology
Clinical features
- The incubation period for infant botulism is 2 to 4 weeks, with a peak incidence from 2 to 4 months
- Infants usually present with constipation, occasionally hypoventilation, followed by hypotonia, drooling, weak cry, generalized muscular weakness ("floppy baby") and hypoventilation; half have upper airway obstruction and cranial nerve palsies (Pediatr Neurol 2005;32:193)
- Has been associated with sudden infant death syndrome (Lancet 1985;1:237, Lancet 1978;1:1273)
- Patients with foodborne botulism generally present with acute, bilateral, symmetric cranial neuropathies
- Symptoms include lack of coordination of eye muscles, double vision, swallowing difficulties and dizziness
- Subsequently there is descending progressive weakness of the extremities and respiratory muscles
- Patients are generally afebrile, alert, and oriented
- Physical exam demonstrates flaccid muscle weakness of tongue, laryngeal muscles, respiratory muscles and extremities
- Without rapid intervention, patients may die of respiratory paralysis or cardiac arrest
Diagnosis
- A high index of suspicion is generally necessary for diagnosis
- Diagnosis is largely clinical through electromyographic studies
- PCR based assays for the bacteria in stool and gastric aspirates or culture are suggestive
- Detection of toxin in stool, blood, contaminated food or the environment is more definitive
- Testing should be performed in public health laboratories, not Level A Laboratories
- These laboratories or the CDC should be contacted for information on specimen transport
- Public health authorities should be notified as epidemiologic investigations are necessary; foodborne botulism is considered a public health emergency
- Of note, infection does not lead to antibody production, and no serologic testing is available
Prognostic factors
- With good supportive care, the death rate is currently < 5%; previously was > 50%
Case reports
- 27 and 28 year old men with botulism from cocaine inhalation (Clin Infect Dis 2006;43:e51)
- 60 year old woman with abdominal pain, dyspnea and diplopia (N Engl J Med 2015;372:364)
Treatment
- Equine derived heptavalent antitoxin (available from CDC) for food borne and wound botulism
- Debridement with antibiotics for wound botulism
- Human Botulism Immune Globulin Intravenous (BabyBig) is administered for infant botulism; generally antibiotics are not given for infant botulism
- Supportive care, especially respiratory support is critical
Differential diagnosis
- Acute motor axonal neuropathy
- Allergic reaction
- Guillain-Barré syndrome
- Miller Fisher syndrome
- Mosquito borne encephalitis
- Poliomyelitis
- Tick paralysis