Botulism – Causes, Symptoms, Diagnosis, Treatment and Ongoing care

Basics

Description

  • Botulism is a muscle-paralyzing illness caused by a neurotoxin made by the bacterium Clostridium botulinum.
  • Characterized by acute onset of bilateral cranial nerve involvement (diplopia, difficulty swallowing or speaking) associated with symmetric descending weakness, intact mental state, no fever, and no sensory dysfunction
  • 7 types of C. botulinum (A–G) are distinguished by their antigenic characteristics. Types A, B, E, and, in rare cases, F, cause disease in humans.
  • Forms include:
    • Foodborne: Caused by ingestion of preform toxin
    • Infant botulism: Caused by ingestion of C. botulinum that produce toxin in the gastrointestinal (GI) tract
    • Wound: Caused by wound infection with C. botulinum that secretes the toxin
    • Aerosolized/inhalational botulinum: Bioterrorism attack potential because of high toxicity; <1 µg is lethal human dose
    • Injection related: Rare
    • Adult colonization botulism: Rare
  • System(s) affected: Neuromuscular; Respiratory; GI
  • Diagnosis is made through history and clinical exam.
  • Laboratory confirmation demonstrates presence of toxin in serum, stool, or wound; or culturing C. botulinum from stool, wound, or food
  • Treatment should not wait for laboratory confirmation.
  • Synonym(s): Sausage poisoning; Kerner disease
  • A purified and diluted form of Type A neurotoxin is used to produce Botox injections.

Epidemiology

Incidence

  • Average of 110 cases of botulism reported annually in US
  • ∼20% of cases are foodborne; 30–40% wound-related; 65% infant botulism
  • Wound botulism incidence increasing due to IV heroin use and cocaine abuse
  • Hidden or intestinal: More common in disorders of the GI tract, such as prior surgery, Crohn disease, or recent antibiotic use
  • Inhalation: Only a single incident involving 3 laboratory workers has been described.

Prevalence

  • Predominant age:
    • Foodborne: Mean age is 46 years; range of 3–78 years
    • Infantile: Mean age of onset 13 weeks, with range of 1–63 weeks
    • Wound: Median age is 41 years with a range of 23–58 years
  • Predominant gender:
    • Foodborne and infantile: Male = Female
    • Wound: Female > Male

Risk Factors

  • Foodborne: Ingestion of home-canned or prepared contaminated foods
  • Infantile: From ingestion of honey or corn syrup; breastfeeding (controversial)
  • Wound: IV drug use (black tar heroin; IM/SC) or “skin popping”

General Prevention

  • Foodborne: Proper handling, processing, preparation (heating), and storage of food; avoid eating food from bulging cans and food that smells/looks spoiled.
  • Infant: Avoid honey before 1 year of age.
  • Wound: Proper wound care
  • Health care providers: Standard precautions
  • If meningitis is suspected in patients with flaccid paralysis, medical personnel should use droplet precautions.
  • Heat potentially contaminated food or drink to an internal temperature of 85°C for at least 5 minutes.
  • After exposure to C. botulinum toxin, clothing and skin should be cleaned with soap and water.
  • Contaminated objects or surfaces should be cleaned with 0.1% bleach solution. All food suspected of contamination should be promptly removed from potential consumers.

Pathophysiology

  • Disease results from hematogenous spread of toxin from mucosal surface (stomach, small intestine) or from an infected wound.
  • The toxin prevents acetylcholine release at presynaptic membranes, blocking neuromuscular transmission in cholinergic nerve fibers.

Etiology

  • Toxin produced by C. botulinum, an encapsulated, anaerobe, gram-positive, spore-forming, rod-shaped bacillus
  • Ingestion of C. botulinum neurotoxins (A, B, and E most common)
  • Foodborne, usually from home-canned vegetables, prepared foods, or foods incubated in anaerobic conditions
  • Infantile from ingestion of spores in environment or occasionally in honey
  • Wound due to contamination with toxin-producing C. botulinum
  • Inadvertent: IM injections of botulinum toxin

Botulism, Clostridium botulinum, Foodborne illness, Clostridium, Botox, cranial nerve involvement, bacterium clostridium botulinum, botox injections, cocaine abuse, sensory dysfunction, wound infection, neurotoxin, antibiotic use, bioterrorism,

Diagnosis

History

  • Foodborne:
    • Incubation: Typically 12–36 hours after toxin ingestion. Rare case as late as 10 days after ingestion.
    • Wound and infant botulism: Incubation time cannot be ascertained.
    • Inhalational: Same as foodborne botulism
  • Adults: Acute onset of symmetric neuropathies. Difficulty in swallowing or speaking, dry mouth. Diplopia, blurred vision, dilated or nonrelated ptosis (drooping eyelids).
  • Symmetric descending, flaccid paralysis in oriented, afebrile patient
  • Respiratory dysfunction
  • Infant botulism: Disease presentation and severity variable:
    • Constipation, shortly followed by weakness, feeding difficulties, descending or global hypotonia, drooling, anorexia, irritability, and weak cry
  • Ask about diet, travel, drug use, and other persons with same symptoms.

Physical Exam

  • General appearance: Oriented, flaccid, may complain of malaise, dizziness, nausea, vomiting
  • Vital signs, afebrile (fever may occur in wound botulism due to secondary infection), normal blood pressure
  • Head, eyes, ears, nose, throat: Dry mouth
  • Chest/lungs: Respiratory muscle weakness, respiratory dysfunction, paralysis
  • Heart: Normal or slow rate
  • Abdomen: Distention, constipation (early sign in infant form); may be absent in wound form
  • Genitourinary: Urinary retention
  • Neurologic:
    • Symmetrical descending weakness beginning with the cranial nerves
    • Ptosis; extraocular muscle paresis; fixed, dilated pupils; dysphagia
    • Infant botulism: Poor muscle tone (loss of head control and facial expression), poor feeding (loss of suck), drooling, feeding difficulties, weak cry
    • Diminished or absent deep tendon reflexes

Diagnostic Tests & Interpretation

Lab

Initial lab tests

  • Laboratory confirmation is done by demonstrating the presence of toxin in serum or stool, or by culturing C. botulinum from stool, wounds, or food.
  • Mouse neutralization assay confirmation:
    • Standard method of diagnosis (1)[B]
    • Available from Centers for Disease Control and some state laboratories; takes ∼4 days for results
  • Routine tests (complete blood count, electrolytes, liver function tests, urinalysis) generally not helpful/show no characteristic abnormalities
  • Cerebrospinal fluid testing: Normal helps differentiate from Guillain-Barré syndrome. Occasionally a borderline elevation in protein is seen.
  • Toxin detected in gastric contents, serum, stool, and suspected food and containers:
    • PCR tests are also available for rapid detection of clostridia in food samples (2)[B].
  • A normal Tensilon test helps to differentiate botulism from myasthenia gravis; borderline can occur in botulism

Imaging

CT or MRI to rule out neurologic pathology

Diagnostic Procedures/Surgery

Electrophysiology testing:

  • Presumptive evidence in patients with negative bioassay studies (3)[C]
  • Brief, small-amplitude motor potential with incremental response on repetitive nerve stimulation

Differential Diagnosis

  • Adult botulisms:
    • Guillain-Barré syndrome
    • Encephalitis, meningitis
    • Tick paralysis
    • Myasthenia gravis
    • Eaton Lambert myasthenic syndrome
    • Cerebrovascular accident: Basilar artery stroke
    • Congenital neuropathy or myopathy
    • Sepsis
    • Hypokalemic periodic paralysis
    • Poliomyelitis
    • Other poisonings (organophosphate, shellfish, Amanita mushrooms, atropine, and aminoglycosides)
    • Miller-Fisher variant of Guillain-Barré syndrome
    • Diphtheritic neuropathy
    • Carbon monoxide intoxication
    • Hypermagnesemia
  • Infant botulism:
    • Sepsis
    • Meningitis
    • Electrolyte–mineral imbalance
    • Reye syndrome
    • Congenital myopathy
    • Leigh disease
    • Werdnig-Hoffman disease

Treatment

Medication

First Line

  • Antitoxin therapy with trivalent A-B-E antitoxin:
    • Call CDC Assistance (770) 488-7100
    • Initiating botulinum antitoxin therapy is primarily based on symptoms and physical examination findings that are consistent with botulism (4)[B].
    • Early administration is important (4)[B].
    • Horse serum derived: Up to 20% reaction incidence. Consider skin testing or pretreatment with steroids or antihistamines.
  • Infantile:
    • Treatment with human botulism immune globulin (BIG-IV or Baby BIG) for botulism types A and B (5)[B]
    • Available only through the California State Health Department (510) 540-2646 or (510) 231-7600
  • Wound:
    • Antitoxin therapy with trivalent A-B-E antitoxin, 1 vial IV and 1 vial IM, repeat in 2–4 hours if persistent symptoms
    • Antibiotics unproven by clinical trial, but widely used and recommended:
      • Penicillin G (3 million units IV q4h in adults)
      • Metronidazole (500 mg IV q8h) for penicillin-allergic patients
    • Vaccine: Pentavalent vaccine available:
      • Efficiency in terrorist attack is unknown
      • Newer vaccines being developed

Second Line

Supportive care, including mechanical ventilation (6)[C]

Pregnancy Considerations

Safety of botulism antitoxin during pregnancy and breastfeeding unknown or controversial (6)

Additional Treatment

Issues for Referral

  • Nutrition: For hyperalimentation and later, tube feeding
  • Physical/occupational therapy: Including swallow evaluation

Additional Therapies

  • Stress ulcer and deep vein thrombosis prophylaxis
  • Pulmonary and physical rehabilitation

Surgery/Other Procedures

Wound excision/debridement

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In-Patient Considerations

Initial Stabilization

Hospital admission with meticulous airway management

Admission Criteria

All suspected cases must be admitted.

IV Fluids

Keep patient well hydrated.

Nursing

  • Prevent decubitus ulcer, IV line infections, other nosocomial infections
  • Before administration of antitoxin, skin testing should be performed for sensitivity.

Ongoing Care

Follow-Up Recommendations

Outpatient follow-up with physical/occupational therapy, nutrition specialist, and psychiatry as needed

Patient Monitoring

  • Pulmonary function testing
  • Cardiorespiratory monitoring

Diet

Nasogastric feedings, if needed

Patient Education

  • Spores destroyed by pressure cooking at 250°F (120°C) for 30 minutes
  • Toxin destroyed by boiling for 10 minutes or cooking at 175°F (80°C) for 30 minutes
  • Avoid honey in 1st year of life.
  • Avoid IV drug use.
  • Do not eat/sample foods that look and smell rotten or come from bulging cans.

Prognosis

  • Delay in administering antitoxin: Most important factor affecting clinical course and outcome (4)[B]
  • Mortality: Overall 7–10%; <5% if infection is treated, but approaches 60% if untreated (6)
  • Mortality for patients >60 years is twice that of younger patients
  • Full recovery may take months.
  • Significant health, functional, and social limitations several years after infection (7)[C]:
    • Recovery follows the regeneration of new neuromuscular connections.
    • 2–8 weeks of ventilator support may be required in more severe cases.
  • Dyspnea with severe ptosis and pupil abnormality has been shown to correlate with severe illness and respiratory failure (8)[C].
  • Increased incubation time has been shown to correlate with better outcomes (8)[C].

Complications

  • Nosocomial infections, including aspiration pneumonia and ventilator-associated pneumonia
  • Hypoxic tissue damage
  • Death

References

1. Lindström M, Korkeala H. Laboratory diagnostics of botulism. Clin Microbiol Rev. 2006;19:298–314.

2. Fach P, Micheau P, Mazuet C, et al. Development of real-time PCR tests for detecting botulinum neurotoxins A, B, E, F producing Clostridium botulinum, Clostridium baratii and Clostridium butyricum. J Appl Microbiol. 2009;107:465–73.

3. Bayrak A, et al. Electrophysiologic findings in a case of severe botulism. J Neurol Sci. 2006;23:49–53.

4. Dembek ZF, Smith LA, Rusnak JM. Botulism: cause, effects, diagnosis, clinical and laboratory identification, and treatment modalities. Disaster Med Public Health Prep. 2007;1:122–34.

5. Arnon SS, Schechter R, Maslanka SE, et al. Human botulism immune globulin for the treatment of infant botulism. N Engl J Med. 2006;354:462–71.

6. O’Brien KK, Higdon ML, Halverson JJ. Recognition and management of bioterrorism infections. Am Fam Physician. 2003;67:1927–34.

7. Gottlieb SL, Kretsinger K, Tarkhashvili N, et al. Long-term outcomes of 217 botulism cases in the Republic of Georgia. Clin Infect Dis. 2007;45:174–80.

8. Witoonpanich R, Vichayanrat E, Tantisiriwit K, et al. Survival analysis for respiratory failure in patients with food-borne botulism. Clin Toxicol (Phila). 2010;48:177–83.

9. Botulism Facts for Healthcare Providers. Accessed 5/30/2010 at http://emergency.cdc.gov/agent/botulism/hcpfacts.asp.

Additional Reading

See Also (Topic, Algorithm, Electronic Media Element)

Food Poisoning, Bacterial

Codes

ICD9

  • 005.1 Botulism food poisoning
  • 040.42 Wound botulism

Snomed

  • 398565003 Infection due to infection due to clostridium botulinum (disorder)
  • 398523009 Foodborne botulism (disorder)
  • 398530003 Wound botulism (disorder)

Clinical Pearls

  • Botulinum antitoxin should be administered as soon as possible; don’t wait for lab results.
  • Medical care providers who suspect botulism in a patient should immediately call their state health department’s emergency 24-hour telephone number.
  • A helpful mnemonic to recall progression of symptoms is the “dozen D’s”: Dry mouth, diplopia, dilated pupils, droopy eyes, droopy face, diminished gag reflex, dysphagia, dysarthria, dysphonia, difficulty lifting head, descending paralysis, and diaphragmatic paralysis (9)

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