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$Unique_ID{BRK03534}
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$Title{Botulism}
$Subject{Botulism Foodborne Botulism Wound Botulism Infant Botulism}
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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
     [phone]

     Centers for Disease Control (CDC)
     1600 Clifton Road, NE
     Atlanta, Georgia  30333
     [phone]

     National Institute of Allergy and Infectious Diseases (NIAID)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

     FDA Office of Consumer Affairs
     5600 Fisher Lane, Rm. 12-A-40
     Rockville, MD  20857
     [phone]

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.