Botulism: Delayed Recognition

Introduction/Background:

Botulism is caused by botulinum neurotoxin (BONT), produced by the anaerobe Clostridium botulinum which causes descending paralysis due to irreversible blockade of pre-synaptic acetylcholine release. Forms include foodborne, infant, wound and iatrogenic. Foodborne botulism is uncommon in the United States and due to improper food preservation, resulting in bacterial contamination, growth and toxin production. Treatment is with botulinum antitoxin (BAT) which binds unbound toxin.

Description:

62-year-old F presented to the Emergency Department (EDP) with GI and neurological complaints 16h after ingesting broccoli cheddar soup (single-serving plastic package, commercially prepared). No PMH or medications. Alert, fully oriented, normal strength. BP 225/106. Sinus tachycarida 120 bpm. Otherwise normal exam. EDP +3h: Neurologist described “heaviness around her eyes and generalized fatigue.” Full visual fields, reactive pupils, conjugated gaze, but significant ophthalmoplegia. Face symmetric, decreased smiling. Dysarthric and dysphonic speech was difficult to understand. Strength decreased: arms 4+/5 and legs 1+/5. Reflexes decreased by 1+. EDP +4h: Transferred to ICU. Desaturation, severe hypercapnia and respiratory acidosis prompted tracheal intubation and mechanical ventilation. The differential diagnosis included Guillan-Barré Syndrome and variants such as Miller Fisher Syndrome, stroke and myasthenia gravis. Myelitis and tick-borne disease seemed improbable. Evaluation included CT head and neck, CBC, chemistries, MRI x2, CT head, EEG and lumbar puncture—all (-) as were cultures, meningitis panel, GM1, GD1b and GC1b antibodies. EDP+5d: Patient paralyzed. History from husband emphasized baseline high-energy state. He mentioned “eyelid heaviness” and diplopia, indicating descending paralysis and the possibility of botulism. CDC and WI Department of Health Services were consulted. Plasma and stool sampling were approved for testing and heptavalent BAT was released by the CDC and infused. EDP +10d: PCR for BONT type A (+). Subsequently, BoNT type A was confirmed by mouse bioassay. EDP +122d: Awake, moving digits, mechanically ventilated in long-term acute care hospital.

Discussion:

Our patient with food-borne botulism presented atypically: Urban, industrialized, sealed plastic container, spoiled taste inconsistent with contamination by C. botulinum as the toxin has no odor or taste. Botulism should be considered early in the differential of descending paralysis and prompt consultation with DPS to request BAT. Only BAT-eligible patients undergo botulism testing.