Botulism

Adult Botulism

Background

• Clostridium botulinum produces toxin that blocks Ach release from presynaptic membrane
  • Ingestion of preformed heat-labile toxin
• Cases due to:
  • Improper (home) canning
  • Black-tar heroin use
  • Wound infection (contaminated wounds, C-section, tooth abscess, sinus infection)
• Symptoms begin 6-48 hr after exposure

Clinical Features

A fully conscious patient with botulism causing weakness of the eye muscles and the drooping eyelids (left) and dilated and non-reactive pupils (right).

Six-week-old infant with botulism, demonstrating marked loss of muscle tone especially in the head and neck.

Infant botulismː despite not being asleep or sedated, he cannot open his eyes or move; he also has a weak cry.

• GI
  • nausea and vomiting, abdominal cramps, diarrhea or constipation
  • Not seen in patients who contract botulism from heroin or contaminated wound
• Neuro
  • Vertigo is common
  • Symmetrical descending weakness leading to flaccid paralysis
  • Cranial nerves and bublar muscles are affected first: diplopia, dysarthria, dysphagia, poor gag reflex
  • Blurred vision and ptosis
  • Decreased deep tendon reflexes
    • Will progress to respiratory depression if not treated
• Anticholinergic signs
  • Decreased salivation: due to cholinergic fiber blockage
    • Dry mouth, painful tongue, sore throat
  • Urinary retention, dry skin/eyes, hyperthermia
• Dilated pupils (in contrast to patients with MG)

Infantile Botulism

Background

• Due to consumption of botulinum spores
  • Ingestion of honey, corn syrup, and vacuum/environmental dust
  • Higher GI tract pH of infants makes them more susceptible
• Most cases occur in <1 yr, 90% occur in <6 mo

Clinical Features

• Floppy Baby Syndrome
  • Loss of facial expression
  • Noticeable neck and peripheral weakness
• GI symptoms
  • Poor feeding
  • Constipation
  • Decreased suckling
• Other
  • Lethargy
  • Weak cry

Differential Diagnosis

Weakness

• Neuromuscular weakness
  • Upper motor neuron:
    • CVA
    • Hemorrhagic stroke
    • Multiple sclerosis
    • Amyotrophic Lateral Sclerosis (ALS) (upper and lower motor neuron)
  • Lower motor neuron:
    • Spinal and bulbar muscular atrophy (Kennedy's syndrome)
  • Spinal cord disease:
    • Infection (Epidural abscess)
    • Infarction/ischemia
    • Trauma (Spinal Cord Syndromes)
    • Inflammation (Transverse Myelitis)
      • Acute flaccid myelitis
    • Degenerative (Spinal muscular atrophy)
    • Tumor
  • Peripheral nerve disease:
    • Guillain-Barre syndrome
    • Toxins (Ciguatera)
    • Tick paralysis
    • Diabetic peripheral neuropathy
  • Neuromuscular junction disease:
    • Myasthenia gravis crisis
    • Botulism
    • Organophosphate toxicity
    • Lambert-Eaton myasthenic syndrome
  • Muscle disease:
    • Rhabdomyolysis
    • Dermatomyositis
    • Polymyositis
    • Alcoholic myopathy
• Non-neuromuscular weakness
  • Can't miss diagnoses:
    • ACS
    • Arrhythmia/Syncope
    • Severe infection/Sepsis
    • Hypoglycemia
    • Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
      • Hypokalemic periodic paralysis
      • Thyrotoxic periodic paralysis
    • Respiratory failure
  • Emergent Diagnoses:
    • Symptomatic Anemia
    • Severe dehydration
    • Hypothyroidism
    • Polypharmacy
    • Malignancy
    • Aortic disease - occlusion, stenosis, dissection
  • Other causes of weakness and paralysis
    • Acute intermittent porphyria (ascending weakness)

Bioterrorism Agents

Category A

• Anthrax
• Smallpox
• Plague
• Tularemia
• Botulinum Toxin
• Viral hemorrhagic fever

Category B

• Ricin
• Brucellosis
• Epsilon toxin
• Psittacosis
• Q Fever
• Staph enterotoxin B
• Typhus
• Glanders
• Melioidosis
• Food safety threats
• Water safety threats
• Viral encephalitis

Category C

• Influenza
• Yellow fever
• Tickborne hemorrhagic fever
• Tickborne encephalitis

Management

Contact CDC Emergency Hotline 1-770-488-7100 for all suspected bioterrorism cases

Supportive Care

• Early ventilatory support
  • Consider intubation when vital capacity <30% predicted or <12cc/kg
• Wound Managment
  • Early wound debreedment with surgical consult.
  • Also exclude Necrotizing fasciitis and coverage with same broad antibiotic coverage

Foodborne Botulism

• Equine Serum Botulism Antitoxin
  • only for patients > 1yo
• Antitoxin obtained through CDC or local Department of Health.

Infant Botulism (<1yo)

• Human-based Botulism IG 100mg/kg IV x 1 dose (BabyBIG)
  • infusion divided into 25mg/kg/hr IV x 15 min followed by 50mg/kg/hr if no allergic reactions
  • Stop infusion after total of 100mg/kg infused
• BabyBIG obtained through CDC or local Department of Health

Inhalational Botulism

• Equine Serum Botulism Antitoxin
  • only for patients > 1yo
• Antitoxin obtained through CDC or local Department of Health

Wound Botulism

• Individualize therapy with ID consultant
• Broad antibiotic coverage same as for Necrotizing fasciitis while awaiting wound cultures

Disposition

• Admit to ICU
• Consider ID Consult

See Also

• Weakness
• Bioterrorism
• Clostridium