Botulism

Botulism

OVERVIEW:

An intoxication producing paralytic disease, caused by neurotoxins of Clostridium botulinum. The toxin prevents acetylcholine release at presynaptic membranes.
• Three forms exist:
• Foodborne botulism
• Infantile botulism
• Wound botulism

System(s) affected: Endocrine/Metabolic, Gastrointestinal, Nervous
Genetics: N/A
Incidence/Prevalence in USA:
• 0.034/100,000 with 75% the infantile form.
• Foodborne - 24 cases/yr
• Infantile - 71 cases/yr
• Wound botulism - 3 cases/yr
Predominant age:
• Foodborne - all ages
• Infantile mean age - 3 months
• Wound - usually young adult
Predominant sex:
• Foodborne and infantile - Male = Female
• Wound - Male > Female

SIGNS AND SYMPTOMS:

• Foodborne
• Nonspecific findings early (nausea, vomiting, malaise, dizziness)
• Dry mouth
• Constipation, urinary retention
• Symmetric descending weakness or paralysis of motor and autonomic nerves, usually beginning with the cranial nerves
• Cranial nerve paralysis (ptosis; extraocular muscle paresis; fixed, dilated pupils; dysphagia)
• Postural hypotension
• Muscle weakness, respiratory paralysis
• Variable deep tendon reflexes
• Afebrile
• Progression over few days
• Infantile
• Constipation - early sign
• Loss of head control
• Loss of suck
• Loss of facial expression and verbalization
• Symmetric descending weakness and cranial nerve paresis similar to foodborne form
• Diminished or absent deep tendon reflexes
• Autonomic dysfunction
• Afebrile
• Usual progression over 2-5 days, can be as short as few hours
• Wound
• Onset 4-14 days post injury
• Findings similar to foodborne botulism
• May be febrile

CAUSES:

• Ingestion of C. botulinum neurotoxins (A, B, and E most common)
• Foodborne usually from home-canned vegetables or prepared foods
• Infantile from ingestion of spores in environment or occasionally in honey
• Wound due to contamination with toxin-producing C. botulinum

RISK FACTORS:

• Foodborne - ingestion of home-canned or prepared foods
• Infantile - ingestion of honey. Breast feeding (controversial)
• IV drug use (e.g., black tar heroin)

DIAGNOSIS

DIFFERENTIAL DIAGNOSIS:

• Guillain-Barre syndrome
• Encephalitis
• Tick paralysis
• Myasthenia gravis
• Basilar artery stroke
• Congenital neuropathy or myopathy
• Sepsis
• Other poisonings (organophosphate, shellfish, Amanita mushrooms, atropine, aminoglycoside)

LABORATORY:

• Routine tests including CSF exam normal
• Confirmation available at the CDC and some state laboratories

Drugs that may alter lab results: N/A
Disorders that may alter lab results: Underlying myoneural disease

PATHOLOGICAL FINDINGS:

Nonspecific

SPECIAL TESTS:

• Stool contains organism and toxin
• Serum toxin present in foodborne form

IMAGING:

N/A

DIAGNOSTIC PROCEDURES:

Electromyogram (EMG) shows characteristic brief, low voltage compound motor-unit, small amplitude, overly abundant action potentials (BSAPs), incremental response to repetitive stimulation

TREATMENT

APPROPRIATE HEALTH CARE:

Inpatient, with maximal monitoring capabilities, especially for respiratory failure

GENERAL MEASURES:

• Meticulous airway management
• Physical therapy with range of motion exercise and assisted ambulation as tolerated
• Prevention of decubiti

SURGICAL MEASURES:

Wound excision debridement

ACTIVITY:

Bedrest initially

DIET:

• Nasogastric feedings if needed
• Fluid restriction if inappropriate antidiuretic hormone (ADH) syndrome

PATIENT EDUCATION:

• When preserving food at home, kill Clostridium botulinum spores by pressure cooking at 250°F (120°C) for 30 minutes
• Toxin can be destroyed by boiling for 10 minutes or cooking at 175°F (80°C) for 30 minutes
• Avoid honey in first year of life

MEDICATIONS

DRUG(S) OF CHOICE:

• Foodborne
• Antitoxin therapy with trivalent A-B-E antitoxin [available at CDC (404) 639-3670 or 639-2888], one vial IV and one vial IM, repeat IV in 2-4 hours if symptoms persist
• Penicillin therapy of unclear value
• Infantile
• Antitoxin therapy not needed
• Penicillin therapy of unclear value
• Enemas may assist in removal of toxin
• Wound
• Antitoxin therapy with trivalent A-B-E antitoxin (available at CDC (404) 639-3753 or 639-2888), one vial IV and one vial IM, repeat in 2-4 hours if persistent symptoms

Contraindications: Aminoglycosides - may potentiate paralysis
Precautions: Serum sickness or hypersensitivity reactions in 20% of antitoxin recipients
Significant possible interactions: N/A

ALTERNATIVE DRUGS:

N/A

FOLLOW UP

PATIENT MONITORING:

Cardiorespiratory monitoring during illness

PREVENTION/AVOIDANCE:

• Avoid giving honey to infants
• Do not eat or taste food from bulging cans, or if food is off-smelling, discard it

POSSIBLE COMPLICATIONS:

• Aspiration pneumonia
• Nosocomial infection
• Hypoxic tissue damage
• Death

EXPECTED COURSE AND PROGNOSIS:

• Foodborne and wound
• Mortality 25% (< 10% under 20 years of age), usually due to delayed diagnosis and respiratory failure
• Full recovery may require months
• Sequelae due to hypoxic insults
• Infantile
• Mortality < 1%
• Extended recovery period and sequelae as above

MISCELLANEOUS

ASSOCIATED CONDITIONS:

N/A

AGE-RELATED FACTORS:

Pediatric: Avoid honey for first year
Geriatric: N/A
Others: N/A

PREGNANCY:

N/A

SYNONYMS:

• Sausage poisoning
• Kerner's disease

ICD-9-CM:

005.1 Botulism

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SEE ALSO (Enter the keywords below into our search box or click on the link):

Food poisoning, bacterial
Tick paralysis

Medical Diagnosis » B » Botulism