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Intestinal botulism

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Disease definition

A rare form of botulism, a rare acquired neuromuscular junction disease with descending flaccid paralysis caused by botulinum neurotoxins (BoNTs), and is due to intestinal colonization by Clostridium botulinum leading to toxin-mediated infection with toxemia. The disease affects infants (infant botulism) and very rarely adults (adult intestinal botulism).

ORPHA:178481

Classification level: Subtype of disorder

Synonym(s):
  • Intestinal colonization botulism
  • Intestinal toxemia botulism
  • Intestinal toxin-mediated botulism

Prevalence: Unknown

Inheritance: -

Age of onset: All ages

ICD-10: A05.1

ICD-11: 1A11.1

UMLS: C1443901

Summary
Epidemiology

Prevalence of intestinal botulism is unknown. So far, nearly 3,400 cases have been reported worldwide, mainly in infants (about 3,350 cases versus 20 cases in adults).

Clinical description

Clinical manifestations are similar to other forms of botulism, in particular those of foodborne botulism (see this term), except for the lack of gastrointestinal symptoms (nausea, vomiting and diarrhea).

Etiology

The disease is caused by botulinum neurotoxins (BoNTs), produced by C. botulinum type A,B,E, or very rarely by neurotoxigenic strains of C. baratii type F and C. butyricum type E, that can temporarily colonize the intestinal tract. Colonization in adults is generally associated with anatomical abnormalities of the gastrointestinal tract or alteration of protective endogenous microflora by broad-spectrum antibiotics following inflammatory intestinal disease or surgery. Colonization in infants is believed to occur because normal competitive microflora has not been fully established. After spore germination and toxinogenesis, the toxin is absorbed into the blood stream and distributed throughout the body, causing the typical manifestations of botulism.

Last update: April 2011 - Expert reviewer(s): Dr Lucia FENICIA
A summary on this disease is available in Français (2011) Español (2011) Deutsch (2011) Italiano (2011) Português (2011) Nederlands (2011) Ελληνικά (2011.pdf)
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