$Unique_ID{BRK03534} $Pretitle{} $Title{Botulism} $Subject{Botulism Foodborne Botulism Wound Botulism Infant Botulism} $Volume{} $Log{} Copyright (C) 1986, 1989, 1990 National Organization for Rare Disorders, Inc. 95: Botulism ** IMPORTANT ** It is possible that the main title of the article (Botulism) is not the name you expected. Please check the SYNONYM listing to find alternate names and disorder subdivisions covered by the article. Synonyms DISORDER SUBDIVISIONS: Foodborne Botulism Wound Botulism Infant Botulism General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Botulism is a form of gastroenteritis due to a bacterial enterotoxin (toxin acting on the intestine). It is a neuromuscular poisoning resulting from Clostridium botulinum toxin. While it may occur in three different forms (foodborne, wound, and infant botulism), the disorder is most often foodborne. In foodborne botulism, toxin which is produced in contaminated food is ingested. Neurotoxin is produced in the body by the growth of C. botulinum in infected tissue in wound botulism, and in the GI tract in infant botulism. Clostridium botulinum spores are highly resistant to heat and they may survive for several hours at 100 C. Exposure to moist heat at 120 C, however, kills the spores. On the other hand, the toxins are readily destroyed by heat. Therefore cooking food at 80 C for 30 minutes protects against Botulism. While home-canned food is the most common source for Botulism, commercially prepared foods have been implicated in about ten percent of the cases. Vegetables, fish, fruits and condiments are the most commonly involved, but beef, milk products, pork, poultry and other foods have also been implicated. Symptoms The onset of Botulism generally occurs twelve to thirty-six hours after the toxin is ingested; however, the incubation period may vary from as little as four hours to a long as eight days. Symptoms include weakness, fatigue, headache, and dizziness. The patient may also experience nausea, vomiting, diarrhea and abdominal pain. Dryness of the mouth and pharynx may occur. Ocular manifestations may include diplopia, which is the perception of two images of a single object or possibly photophobia, an abnormal intolerance of light. Later in the course of the disease, there may be constipation, difficulty in swallowing known as dysphagia, and the patient's throat may be constricted. Difficulties in breathing may also be present. Clinical manifestations may include the following conditions: 1. Mydriasis (dilation of the pupil) 2. Ptosis (drooping of the eyelid) 3. Nystagmus (an involuntary rapid movement of the eyeball) 4. Tachycardia (rapid heartbeat) 5. Irregular respiration 6. Cheyne-Stokes respiration (breathing characterized by rhythmic waxing and waning of the depth of respiration which may be accompanied by periods of apnea) 7. A tongue that is swollen and coated 8. Hyporeflexia (weakening of the reflexes) Botulism may also produce progressive muscular paralysis, possibly abdominal distention characterized by the absence of normal intestinal sounds, and urinary retention. Fever is usually minimal and may, in fact, be absent. Wound botulism is characterized by the same neurological symptoms. as foodborne botulism; however, the patient experiences no gastrointestinal symptoms nor is there any evidence implicating food as the cause. The skin must be carefully checked for a wound. Infant botulism is seen most often in infants between the ages of two and three months. Constipation is initially present in approximately two-thirds of cases. This may be followed by neuromuscular paralysis. The severity of the disease varies. Infants afflicted with infant botulism have generally been exposed to foods other than milk contaminated with spores which are common in the environment. Cases have been related to the ingestion of honey, vacuum cleaner dust and soil which contains C. botulinum. Causes Botulism is the result of ingestion and absorption of toxin which is produced by the Clostridium botulinum bacillus. While there are seven distinct types of the toxin, human poisoning is usually caused by Type A, B, E, or F. Wounds may be infected with Clostridium, but this occurrence is rare. Unlike foodborne botulism, infant botulism results from ingestion of the botulinal spores which then grow and produce within the body. Neuromuscular transmission in cholinergic nerve fibers is blocked by the toxin. Affected Population Botulism resulting from Types A and B Clostridium botulinum may occur worldwide. While Type A is the most common in the United States, it is most predominant west of the Mississippi River. Type B is found more often in the Eastern states, and Type E is more prevalent in Alaska and the Great Lakes area. Type E also frequently occurs in northern latitudes and Japan. Therapies: Standard It is essential that both home canned and commercially canned foods be prepared properly, and food must be adequately heated before serving to prevent botulism. Food which shows any sign of spoilage should be discarded. Unabsorbed toxin may be eliminated by induction of vomiting, gastric lavage, and purgation. Since respiratory impairment and its complications may be life threatening, patients should be hospitalized and closely supervised. Trivalent antitoxin (A, B, E) is available from the Centers for Disease Control in Atlanta, GA. They also supply a polyvalent antitoxin for specific outbreaks which are due to Types C, D, or F botulism. Treatment should be initiated as soon as possible. However, the risks of treatment must be weighed against potential benefits. The antitoxins are made from horse serum and there is the possibility of anaphylaxis or serum sickness. It may even be beneficial to begin treatment even several weeks after ingestion of the toxin. While the use of antitoxin does not reverse preexisting neurological impairment or the binding of already bound toxin, it may possibly slow and halt further progression of the disease. Guanidine has been advocated in the treatment of some patients affected with botulism. However, reported results have been inconclusive and thus far the effectiveness of the drug remains unproven. Therapies: Investigational The Office of Orphan Products Development awarded a New Grant Award for 1990 to Dr. Stephen S. Amon of the California Department of Health Services, Berkeley, CA, for clinical trials of Human Botulism Giobulin (BIG). This disease entry is based upon medical information available through October 1990. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Botulism, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, Georgia 30333 (404) 639-3534 National Institute of Allergy and Infectious Diseases (NIAID) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 FDA Office of Consumer Affairs 5600 Fisher Lane, Rm. 12-A-40 Rockville, MD 20857 (301) 443-4903 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Eds.: W. B. Saunders Co., 1988. P. 1633-4, 63, 66. THE MERCK MANUAL 15th ed.: R. Berkow, et al: eds; Merck, Sharp & Dohme Research Laboratories, 1987. Pp. 783-4.