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Botulism

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NORD is very grateful to Agam Rao, MD, Division of Foodborne, Waterborne, and Environmental Diseases, Centers for Disease Control, for assistance in the preparation of this report

Synonyms of Botulism

Disorder Subdivisions

General Discussion

Botulism is a rare but serious paralytic disease caused by a bacterial toxin usually produced by the bacterium Clostridium botulinum. There are four generally recognized naturally-occurring types; foodborne, wound, infant, and, rarely, adult intestinal colonization. Iatrogenic and inhalational botulism may also occur. Foodborne botulism is caused by eating foods that contain botulinum toxin. Wound botulism occurs when C. botulinum spores germinate and produce toxin in a contaminated wound or abscess. The most common form of botulism in the United States, infant botulism, is caused when ingested C. botulinum spores colonize and subsequently produce toxin in the intestines of affected infants. In rare instances, C. botulinum intestinal colonization and toxin production have also occurred among adults with anatomical or functional bowel abnormalities. Additionally, botulism has infrequently occurred after intramuscular injection of botulinum toxin for treatment of certain dystonias and other disorders. Finally, inhalational botulism, though not naturally-occurring, was reported among three German laboratory workers who inadvertently inhaled aerosolized toxin, and could potentially occur after a deliberate aerosolization of toxin in a bioterrorism event.

Any case of foodborne or unexplained botulism is considered to be a public health emergency because of the potential for toxin-containing foods to injure others who eat them and because of the potential misuse of botulinum toxin as a biological weapon. State and local public health officials by law must be informed immediately whenever botulism is suspected in a human patient.

Symptoms

The incubation period for foodborne botulism is usually 12 - 36 hours but may range from a few hours to 10 days. Symptom onset would likely occur faster in an inhalational botulism scenario. Symptoms can range from mild to severe. The classic medical triad of botulism is a flaccid descending paralysis in a patient without fever (afebrile) and with a clear sensorium (alert and able to respond to questions). Fever might occur after a secondary infection (e.g., aspiration pneumonia).

The progression of illness is similar among patients with different types of botulism (foodborne, wound, inhalational) except that foodborne botulism patients often experience gastrointestinal symptoms such as nausea, vomiting, and diarrhea, that precede the onset of neurologic illness.

The typical neurologic presentation of botulism is a symmetric (same on both sides of the body), descending flaccid paralysis that begins in the cranial nerves and progresses to include proximal and then distal extremity muscle groups. Severe illness can result in respiratory paralysis and death. Some patients also experience abdominal pain or cramping, constipation or ileus (paralytic obstruction of the intestines), and urinary retention. Sensory deficits or paresthesias are uncommon, and mental status typically remains clear provided the patient is adequately oxygenated.

Cranial nerve deficits are usually bilateral, typically precede respiratory impairment or extremity weakness. Eye signs or symptoms may include double vision (diplopia) and/or blurred vision, impaired functioning of the muscles of the eyes (ophthalmoplegia), droopy eyelids (ptosis), dilation of the pupil (mydriasis), and depressed or loss of pupillary constriction to light. Other findings may include difficulty speaking (dysphonia) or slurred speech (dysarthria), difficulty in swallowing (dysphagia), a dry mouth and very dry or sore throat. Some individuals have a tongue that appears swollen or "coated" due to its dryness. The gag reflex may be suppressed.

Affected individuals may experience generalized muscle weakness, often progressing rapidly from the head to involve the neck, arms, chest, and legs. The weakness is usually the same on both sides of the body (symmetric), descending, and spreads from proximal to distal muscle groups. Weakening of the deep tendon reflexes (hyporeflexia) or complete loss of reflexes (areflexia) may also be present. Difficulty breathing may also be present, and may progress to respiratory failure from pharyngeal and upper airway muscle paralysis as well as paralysis of the diaphragm and accessory muscles of respiration.

Wound botulism is characterized by the same neurological symptoms as foodborne botulism. However, the affected individual experiences no gastrointestinal symptoms and no food can be implicated as the cause. The skin must be carefully checked for wounds. In the United States, wound botulism most commonly occurs among users of injection drugs, particularly black tar heroin. Wound botulism may rarely occur after traumatic injury involving contamination with soil, or potentially after surgery. A fever due to infection from other bacteria may be present.

Infant botulism generally affects infants under 52 weeks of age. The action of the toxin in the body produces constipation, weakness (notably of gag, cry, suck, and swallow), loss of muscle tone, and ultimately, limp (flaccid) paralysis. The severity and rapidity of onset of the disease varies greatly among affected infants. In the absence of complications, recovery is complete.

Causes

Foodborne botulism is the result of ingestion and absorption of toxin that is produced by Clostridium botulinum in contaminated foods. Wound botulism is caused by toxin produced from a wound infected with Clostridium botulinum. Unlike foodborne and wound botulism, infant botulism and adult intestinal colonization botulism result from ingestion of the bacterial spore(s) that grow and produce botulinum toxin within the large intestine. Honey is the one identified and avoidable food reservoir of these spores for infant botulism. After testing over the years of hundreds of foods, beverages and other items placed in infants' mouths with negative results, it was concluded that the source of spores for most infant botulism patients is unknown but may be from ingestion of microscopic dust particles on which the spores travel.

Botulinum toxin causes weakness and loss of muscle tone because it blocks the nerve ending's ability to signal the linked muscle to contract.

The bacterium Clostridium botulinum is found in soils and marine sediments throughout the world. In the United States, foodborne botulism has been associated primarily with home-canned foods, particularly vegetables, and with Alaska Native foods, especially fish.

Clostridium botulinum typically produces one of seven distinguishable toxin types, arbitrarily designated by the letters A through G,. Botulism in humans is usually caused by toxin types A, B, and E, and rarely F. Most foodborne botulism is caused by contaminated home-canned foods, though outbreaks also occur from commercial foods, restaurant foods, or improper food handling practices. Foodborne botulism may also be associated with the ingestion of baked potatoes that have been stored (for extended periods of time) in aluminum foil at room temperature and with certain condiments, such as onion or garlic, stored in oil. Outbreaks of Type E are usually associated with consumption of preserved, uncooked foods prepared from aquatic (freshwater or marine) fish or mammals. Types A and B are the principal causes of infant and wound botulism.

Affected Populations

Infant botulism occurs globally and is the most common form of human botulism in the United States. Patients diagnosed with botulism demonstrated a predominance of toxin type A west of the Rocky Mountains and type B east of the Rocky Mountains, while type E is more prevalent in the Pacific Northwest, Alaska and the Great Lakes area. Type E also frequently occurs in northern latitudes and Japan. Between 1976 and 1984, there were 124 outbreaks of foodborne botulism reported to Centers for Disease Control and Prevention (CDC).

In 2010, 112 cases of botulism (all forms) were reported to CDC. Of these, there were 85 cases of infant botulism, 9 cases of foodborne botulism, 17 cases of wound botulism, and one case of unknown type. The 85 reported cases of infant botulism resulted from toxin type A (30 cases, 35%), toxin type B (54 cases, 64%), and toxin type F (1 case, 1%). There were two foodborne outbreaks with two cases associated with seal blubber and 2 cases associated with an unknown Alaska native food. In 2010, no deaths from botulism were reported.

Related Disorders

Symptoms of the following disorders can be similar to those of botulism. Comparisons may be useful for differential diagnosis.

Eaton-Lambert syndrome is a neuromuscular disorder that may be an autoimmune disease. Major symptoms include muscle weakness and fatigue especially of the pelvic and thigh muscles. Other symptoms may include dryness of the mouth, impotence, pain in the thighs, and a pricking, tingling or creeping sensation on the skin (paresthesias) around the affected areas. (For more information on this disorder, choose "Eaton-Lambert" as your search term in the Rare Disease Database.)

Guillain-Barre syndrome (acute idiopathic polyneuritis) is a rare, rapidly progressive disorder resulting from inflammation of the nerves (polyneuritis) and resulting in paralysis. Although the precise cause of Guillain-Barre syndrome is unknown, a viral infection or Campylobacter gastroenteritis precedes the onset of the syndrome in about half of the cases. This has led to the theory that Guillain-Barre syndrome may be an autoimmune disease (caused by the body's own immune system). Damage to the covering of nerve cells (myelin) and nerve axons (the extension of the nerve cell that conducts impulses away from the nerve cell body) results in delayed nerve signal transmission. There is a corresponding weakness in the muscles that are supplied with nerve impulses (innervated) by the affected nerves. (For more information on this disorder, choose "Guillain-Barre Syndrome" as your search term in the Rare Disease Database.)

Myasthenia gravis is a chronic neuromuscular disease characterized by weakness and abnormally rapid fatigue of muscles, particularly those that are controlled by the brain stem (bulbar-innervated). The symptoms of myasthenia gravis typically improve following a period of rest. Any muscle may be affected by this disorder. However, the muscles around the eyes (extraocular) and those used in swallowing are most frequently affected by myasthenia gravis. (For more information on this disorder, choose "Myasthenia Gravis" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis

The clinical diagnosis of botulism may be made based upon a thorough clinical evaluation and a detailed patient history. The mouse bioassay is the "gold standard" method for laboratory confirmation of the clinical diagnosis and may demonstrate the presence of botulinum toxinin serum, gastric fluid stool), or food (foodborne botulism) specimens. Botulism can also be confirmed by culture of C. botulinum organisms from gastric fluid), stool), or a contaminated wound (wound botulism).

Prevention

It is essential that both home-canned and commercially-canned foods be prepared properly to prevent the production of botulinum toxin. Food that shows any sign of spoilage should be discarded.

The following link provides information about proper techniques for canning:

http://nchfp.uga.edu/publications/publications_usda.html

Clostridium botulinum spores are highly resistant to heat and may survive for several hours at tempatures of 100 C. Exposure to heat at 120 C destroys C. botulinum spores. In contrast to spores, botulinum toxin is easily inactivated by heat; thus, thoroughly heating food at 85 C or boiling is recommended. While home-canned food is the most common source for botulism, commercially prepared foods have been implicated as well. Vegetables, fish, fruits and condiments are the most commonly implicated foods; however beef, dairy products, pork, poultry, and other foods have also been implicated. To reduce the risk of infant botulism, honey should not be fed to infants less than 12 months of age.

Treatment

Since respiratory impairment and its complications may be life-threatening, affected individuals should be hospitalized, closely supervised and promptly treated with antitoxin. Botulism is a public health emergency and physicians should immediately report a suspect case to their state health department. Rapid notification of states ensures that the appropriate health authorities can work to identify or prevent other cases of botulism. State health departments may then contact Centers for Disease Control and Prevention (CDC) to discuss appropriate testing and treatment of suspected patients.

CDC provides additional information about botulism online at:

http://www.cdc.gov/nczved/divisions/dfbmd/diseases/botulism/

Intravenous equine antitoxin, administered as early as possible in the course of illness, is the only specific treatment available for foodborne and wound botulism. Antitoxin will not reverse established neurological damage, but may prevent the progression of disease. Heptavalent botulinum antitoxin that covers all 7 known botulinum toxin types and is despeciated is available from CDC. Treatment should be initiated as soon as possible once botulism is suspected. However, the risks of treatment must be weighed against potential benefits. The antitoxin is made from horse serum and there is a possibility of adverse reactions including anaphylaxis or serum sickness in some individuals.

For the treatment of infant botulism, physicians may obtain Botulism Immune Globulin Intravenous (Human) (BabyBIG®) from the California Department of Health Services, Infant Botulism Treatment and Prevention Program (IBTPP) following clinical consultation with the on-call Program physician. BabyBIG® has been shown in clinical studies to substantially reduce the length and cost of hospital stay for infants treated within seven days of hospital admission. For suspected cases of infant botulism, the attending physician should first contact their state health department, and then may contact IBTPP at (510) 231-7600 (24 hours, 7 days) to request BabyBIG®. The IBTPP webpage at www.infantbotulism.org has additional information.

Antibiotic therapy should be avoided in the treatment of foodborne or infant botulism.

Historically, guanidine was used to treat some patients affected with botulism, but it is no longer used.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Botulism Resources

Organizations:

References

TEXTBOOKS
Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:287-89.

Abrutyn E. Botulism. In: Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:904-05.

Berkow R, ed. The Merck Manual-Home Edition. Whitehouse Station, NJ: Merck Research Laboratories; 1997:516-18.

Bartlett JG. Botulism. In: Bennett JC, Plum F, eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:1635-36.

Bleck TP. Clostridium Botulinum. In: Mandell GL, et al., eds. Mandell, Douglas and Bennett's Principles and Practice of Infectious Diseases. 4th ed. New York, NY: Churchill Livingstone Inc; 1995:2178-82.

JOURNAL ARTICLES
Sobel, J. Botulism. Clin Infect Dis. 2005; 41(8):1167 - 73.
Krishna S, et al. Infant botulism: case reports and review. J Ky Med Assoc. 2001;99:143-46.

Klein AW. Complications and adverse reactions with the use of botulinum toxin. Semin Cutan med Surg. 2001;20:109-20.

Arnon SS, et al. Working Group on Civilian Biodefense. Botulinum toxin as a biological weapon: medical and public health management. JAMA. 2001;285:1059-70.
Muensterer OJ. Infant botulism. Pediatr Rev. 2000;21:427.

Byrne MP, et al. Development of vaccines for the prevention of botulism. Biochemie. 2000;82:955-66.

Shapiro RL, et al. Botulism in the United States: A clinical and epidemiologic review. Ann Intern Med. 1998; 129(3):221 - 8.

Angulo FJ, et al. A large outbreak of botulism: the hazardous baked potato. J Infect Dis. 1998;178:172-77.

Shapiro RL, et al. Botulism surveillance and emergency response: A public health strategy for a global challenge. JAMA. 1997;278(5):433 - 5.

Kakinuma H, et al. Application of nested polymerase chain reaction for the rapid diagnosis of infant botulism type b. Acta Paediatr Jpn. 1997; 39:346-48.

Yamakawa K. Emergence of clostridium botulinum type b-like nontoxigenic organisms in a patient with type b infant botulism. J. Clin Microbiol. 1997; 35:2163-64.

Maselli RA. Cluster of wound botulism in california:clinical, electrophysiologic, and pathologic study. Muscle Nerve. 1997; 20:1284-95.

Zouari N, et al. Importance of electromyography in the diagnosis of botulism. Neurophysiol Clin. 1997;27:220-26.

INTERNET
National Botulism Surveillance. National Surveillance Team - Enteric Diseases Epidemiology Branch. Centers for Disease Control and Prevention. http://www.cdc.gov/nationalsurveillance/botulism_surveillance.html. Updated December 14, 2011. Accessed May 29, 2012.

Report last updated: 2012/06/01 00:00:00 GMT+0