Botulism
Botulism: Excerpt from The 5-Minute Pediatric Consult
Sheila M. Nolan, MD
Botulism - BASICS
Botulism - description
- An illness produced by neurotoxins elaborated by Clostridium botulinum, which causes an acute, descending, flaccid paralysis
- The neurotoxin may be ingested or absorbed from infected wounds, or ingested spores germinate, producing toxin.
- There are 3 types of illness:
- In infant botulism, ingested spores germinate and colonize the infant’s colon and elaborate toxin.
- In adults, the patient ingests preformed toxin while eating improperly prepared or stored foodstuffs.
- In wound botulism, spores germinate in an infected wound and toxin is absorbed.
Botulism - general prevention
- Botulinum toxin is heat labile; 5 minutes of boiling will destroy the toxin.
- Home-canned foods should be boiled for ≥10 minutes before serving.
- Spores are more resistant to heat. Home canners must use temperatures well above boiling to destroy spores effectively (120°C for 30 minutes). Pressure cookers are needed to achieve these conditions.
Botulism - epidemiology
- Infants are usually white, breastfed, and from middle-class families.
- There often is a history of a recent change in feeding practice (addition of formula or solids or changing from breast to bottle feeding)
- Honey seems to be a particularly contaminated food and has been implicated in California. Corn syrup has also been reported to contain botulinum spores but much less frequently than honey and has been associated with significantly fewer cases of infant botulism.
- Breastfed infants get ill at an older age than do bottle-fed infants; all cases of sudden infant death syndrome (SIDS) associated with infant botulism have been in bottle-fed infants.
- Food-borne cases are usually associated with the use of home-processed foods—especially vegetables, fruits, and condiments.
Botulism - incidence
- Infant botulism occurs in the 1st year of life, with >95% of cases reported in the 1st 6 months.
- Intestinal botulism is the most common form of human botulism in the US, with >100 cases reported annually.
- Wound botulism is very rare.
Botulism - prevalence
- Cases are seen more frequently in rural and suburban areas.
- Most cases have been reported in California, Utah, and Pennsylvania.
Botulism - risk factors
Infants who have <1 bowel movement per day may be at increased risk.
Botulism - pathophysiology
- Neurotoxin is taken up by nerve endings and irreversibly blocks acetylcholine release in peripheral cholinergic synapses.
- Cranial nerves are usually affected first and most severely, leading to difficulty swallowing and loss of airway-protective reflexes. Respiratory failure develops.
- Botulinum toxin does not cross the blood–brain barrier; therefore, the sensorium remains clear.
- Recovery occurs with the regeneration of terminal motor neurons and the formation of new motor end plates.
- Infants are particularly prone to colonic colonization with C. botulinum. When foods other than breast milk are introduced in breast-fed infants, changes in flora may be especially important.
Botulism - etiology
C. botulinum, the etiologic agent, is a Gram-positive, spore-forming, obligate anaerobic bacteria that is found in soil throughout the world.
Botulism - DIAGNOSIS
Botulism - signs & symptoms
Botulism - history
- Usually constipation, with a progressive course of lethargy, weakness, and poor feeding
- Occasionally, progression may be quite rapid, and the abrupt onset of lethargy and weakness may suggest the diagnosis of bacterial sepsis or meningitis.
- Food-borne cases result in complaints of emesis in ~50% of patients.
- There may initially be complaints of diarrhea followed by constipation.
- The incubation period from ingestion to the onset of symptoms is usually 18–36 hours (range, a few hours to several days).
- Patients complain of weakness and dry mouth.
- Visual complaints include blurry vision, loss of accommodation, and diplopia.
- Patients may complain of dysphagia or dysarthria.
- Patients may have urinary retention.
- Fever is absent.
- Within 3 days, there is the onset of the characteristic descending, symmetrical paralysis. The cranial nerves are usually affected 1st.
- Mentation is clear, except for understandable anxiety and agitation.
- Wound botulism:
- Has an incubation period of 4–14 days
- Fever may or may not be present.
- Patients often report constipation, but rarely nausea or vomiting.
- They may complain of unilateral sensory changes and of purulent discharge from the wound.
Botulism - physical exam
- Older children and adults:
- Often appear alert and are afebrile
- Ptosis, extraocular palsies, and fixed and dilated pupils are often the 1st signs of descending paralysis.
- Loss of airway-protective reflexes and respiratory muscle weakness leads to respiratory failure.
- The triad of bulbar palsies, a lucid sensorium, and the absence of fever should prompt one to consider strongly a diagnosis of botulism.
- Infant botulism:
- Presents in a similar way
- Patients are usually afebrile.
- They are usually weak, with decreased spontaneous activity at presentation.
- They have an expressionless (masklike) face, ptosis, a weak cry, poor head control, and generalized weakness and hypotonia.
- Pupils:
- Often midposition initially and may be at least weakly reactive
- Pupillary response is fatigable.
- In many cases, pupils become fixed and dilated for a period.
- Except for the symmetric, descending paralysis, the remainder of the physical examination is normal.
- Signs of autonomic instability include unexpected fluctuations in skin color, BP, and heart rate.
- Physical examination trick:
- In infants, early in the course of the disease, pupillary and corneal reflexes may fatigue easily.
Botulism - tests
Requirements for testing:
- Most tests for toxin and cultures are conducted by state health departments.
- The most common test performed is an assay for botulinum toxin in stool.
- Specimens must be shipped in sealed, break-proof, and leak-proof containers. Even small amounts of toxin, if inhaled or ingested, can lead to disease.
- Suspect foods should be shipped refrigerated and in their original containers if possible.
Botulism - lab
- Tests for the presence of toxin or the organism can be conducted on patient samples (serum, gastric aspirates, feces, or wound exudate) or suspected foodstuffs.
- Anaerobic cultures of a wound or the GI tract may yield the organism.
Botulism - imaging
EEG, MRI, and CT are nonspecific and usually normal in the absence of any complications.
Botulism - diag proced-surgery
Electromyography (EMG) shows a characteristic pattern of brief-duration, sharp-amplitude, overly abundant motor unit action potentials (brief short-acting potentials).
Botulism - differencial diagnosis
- Infections:
- In noninfants, bacterial sepsis, meningitis, poliomyelitis, tick paralysis, and diphtheric polyneuritis
- In infants, sepsis and meningitis may present in a similar way.
- Absence of fever and a clear sensorium make sepsis and meningitis less likely.
- Neurologic:
- Myasthenia gravis usually spares the pupillary response, whereas it is fatigable in botulism, if not absent.
- In Werdnig-Hoffman disease (type I spinal muscle atrophy), facial muscles are spared.
- Toxins: Drug ingestions may lead to weakness and lethargy.
Botulism - TREATMENT
Botulism - initial stabilization
Good supportive care with emphasis on respiratory support, including intubation and mechanical ventilation when needed, is the most important consideration in emergency therapy.
Botulism - general measures
- All patients with suspected botulism should be admitted to the hospital and have continuous monitoring of their heart rate, respiratory rate, and oxygenation, as well as frequent assessment of their respiratory effort and airway-protective reflexes.
- The mainstay of therapy is meticulous supportive care. Particular attention is paid to respiratory and nutritional needs.
- Endotracheal intubation may be necessary both for patients with frank respiratory failure and when airway-protective reflexes are lost.
- Wounds should be explored and débrided, and anaerobic cultures should be obtained.
- Cases of suspected toxin ingestion should be treated early with induced emesis and/or gastric lavage in an attempt to decrease toxin exposure.
- All cases should be reported to the state health department and the Centers for Disease Control (CDC), Atlanta, Georgia.
- Supportive care should be continued until the patient is able to be weaned from respiratory support and begin PO feedings.
Botulism - medication
- Antibiotics are not helpful in infant botulism:
- In suspected infant botulism, aminoglycoside antibiotics (e.g., gentamicin) should be avoided, as they may produce an abrupt worsening of the weakness and ensuing respiratory failure.
- Prompt recognition of infant botulism and early treatment with human IV botulism immune globulin has been shown to decrease time to recovery and hospital discharge. The FDA approved the use of human intravenous botulism immune globulin for the treatment of infant botulism in 2003.
- Equine antitoxin is not recommended for infant botulism.
- Antibiotics are indicated only for documented complications such as pneumonia.
- Cathartics are not beneficial, and enemas may cause colonic distention and increased toxin absorption.
- Cases of botulism resulting from ingested toxin or wound infection:
- Should be treated with botulism equine trivalent antitoxin, available from the CDC
- Antitoxin should not be administered to asymptomatic individuals who have only eaten suspect foods.
- Wound botulism should be treated with IV penicillin G 250,000 U/kg/d.
Botulism - FOLLOW UP
Botulism - prognosis
- Food-borne botulism carries a mortality rate of 20–25%. This rate is lower in patients <20 years old (10%).
- Patients with a shorter incubation period usually have more severe involvement and a worse prognosis, probably related to an increased amount of toxin ingested.
- If recognized early and treated aggressively, botulism carries a good prognosis, and complete recovery can be expected. Fatigability may persist for up to 1 year.
- Infant botulism has an estimated mortality rate of <5% in hospitalized patients. Complete recovery can be expected when disease is recognized early and treated appropriately.
Botulism - complications
- The most serious and fatal complication is respiratory failure due to paralysis of the respiratory muscles.
- Bulbar dysfunction in infant botulism may lead to dehydration before presentation.
- The loss of airway-protective reflexes can lead to aspiration and pneumonia.
- Constipation and urinary retention may precede the onset of paralysis and may complicate later management as well. Cases of severe Clostridium difficile enterocolitis with hypovolemia, hypotension, and prolonged ICU stays have been reported in infants with botulism.
- The earliest symptoms in adults and older children may be visual changes, including blurred vision, loss of accommodation, and diplopia.
- Syndrome of inappropriate secretion of diuretic hormone and urinary tract infections have been reported in infants with infant botulism.
Botulism - bibliography
- Arnon SS, Schechter R, Maslanka SE, et al. Human botulism immune globulin for the treatment of infant botulism. N Engl J Med. 2006;354(5):462–467.
- Infant botulism—New York City, 2001–2002. JAMA. 2003;289:834–836.
- Muensterer OJ. Infant botulism. Pediatr Rev. 2000;21:427.
- Passaro DJ, Werner SB, Mcgee J, et al. Wound botulism associated with black tar heroin among injecting drug users. JAMA. 1998;279:859–863.
- Shapiro RL, Hatheway C, Swerdlow DL. Botulism in the United States: A clinical and Epidemiologic review. Ann Intern Med. 1998;129:221–228.
Botulism - CODES
Botulism - icd9
005.1 Botulism
Botulism - FAQ
- Q: Can infant botulism recur?
- A: True recurrence in infant botulism has not been documented.
- Q: Should antitoxin be given to persons who have ingested food that they think might be contaminated with botulinum toxin?
- A: Because the antitoxin carries a significant risk of serum sickness, it should be given only to persons with neurologic symptoms.
- Q: Where is antitoxin obtained?
- A: Antitoxin may be obtained from the Centers for Disease Control and Prevention, Atlanta, Georgia; (404) 639-3753 (days), (404) 639-2888 (nights).
- Q: Where is human IV botulism immune globulin obtained?
- A: Human IV botulism immune globulin, which is produced from pooled human plasma from screened individuals, may be obtained from the California Department of Health Services Infant Botulism Treatment and Prevention Program; (501) 540-2646.
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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