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6. Botulism: “I don’t need to talk, but I do need to swallow!”
Keywords
BotulismWound botulismParalysisBOTOXClostridium botulinumCase
Diplopia, Dysarthria, and Dysphagia
Pertinent History
A 30-year-old male with a history of type 1 diabetes presents to the Emergency Department with 3 days of worsening blurred and double vision, difficulty speaking, and progressive dysphagia. He states that he initially noted a mild, dull diffuse headache and blurred vision for which he was evaluated at an outside hospital. During that visit, he was treated for a migraine headache with improvement of his symptoms and discharged. However, while the headache had resolved, his blurred vision progressed to double vision, and he noted difficulty in formulating words and swallowing both solids and liquids, with associated hoarseness of his voice. At present, his voice is muffled, his speech is slurred almost like he is intoxicated, and he has pooling secretions in his oropharynx. No others around him have been sick. He did not eat anything unusual. He denies recent travel, wounds, or environmental exposures.
Past Medical History (PMH)
Type 1 diabetes
Social History (SH)
Former IV drug user, but has “been clean for sometime now”
Pertinent Physical Exam
Vital Signs:
BP 165/90, HR 90, RR 16, Temperature 99.4 ° F/37.4 C
Except as noted below, the findings of the complete physical exam are within normal limits.
Head, eyes, ears, nose, throat (HEENT):
Bilateral ptosis and ophthalmoplegia including cranial nerves 3 and 6, with a weak gag reflex and pooling secretions in the oropharynx. He has an asymmetrical rise in his palate when trying to say “aah.”
Neurological Exam:
Symmetrically decreased 1/4 upper extremity reflexes, and 2/4 lower extremity reflexes. He is notably ataxic on ambulation but with a normal motor and sensory exam otherwise.
Pertinent Diagnostic Testing
CBC, serum chemistry, liver function testing, urinalysis – within normal limits
Negative inspiratory force: −40 cm H2O (normal −80 to −100 cm H2O)
Vital capacity: 2.8 liters (3–5 liters)
ED Management
Our differential diagnosis included Guillain–Barre syndrome, specifically the Miller–Fisher syndrome, as well as neuromuscular junction disorders such as myasthenia gravis, infiltrative central nervous system processes (i.e., lymphoma, metastatic cancer, neurosarcoidosis), Lyme disease, botulism, and tick paralysis. Neurology was consulted with continued airway monitoring in the ED. An immediate MRI of his brain was unremarkable, as were the results of a lumbar puncture. He was subsequently admitted to the ICU for monitoring given concerns for impending respiratory failure.
Test results for HIV, Lyme disease, acetylcholine receptor antibody, and ganglioside antibody panel were negative. He was empirically started on IVIG for presumed Miller–Fisher variant Guillain–Barre syndrome while awaiting other studies. Further inpatient questioning revealed that he had, in fact, recently relapsed and started using IV heroin again, sharing needles with multiple persons. He had initially withheld this information to prevent his family from finding out about his addiction. Shortly after admission, he was intubated for inability to handle secretions and impending respiratory failure. Given the new history, concerns were focused on the possibility of wound botulism.
Learning Points
Priming Questions
- 1.
What are the classic presenting features of botulism?
- 2.
What historical clues can help you delineate the diagnosis?
- 3.
What diagnostic workup and treatment should be provided?
- 4.
What are the characteristics of some important members of the differential diagnosis?
Introduction/Background
- 1.
Botulism is a rare, neurotoxin-mediated illness produced by the Gram-positive, anaerobic spore-forming bacilli Clostridium botulinum [1, 2]. Four different categories are described for surveillance purposes, including foodborne, wound, infant, and others.
Wound Botulism: The bacteria, C. botulinum, infects the wound and produces the toxin, which is responsible for the neurological symptoms. This is most often associated with the intramuscular or subcutaneous injection of black tar heroin.
Infant Botulism: Accounts for 80% of laboratory-confirmed botulism cases. Infants less than 1 year of age may become infected by ingesting germinated C. botulinum spores, which then elicit neurotoxin while in the gastrointestinal tract. The classically described association is with the ingestion of honey; however, the majority of infant botulism cases are actually caused by swallowing dust particles, which contain C. botulinum spores.
Foodborne Botulism: In contrast, foodborne botulism seen in adults and children greater than 1 year of age is acquired by ingestion of preformed toxin, as the matured gastrointestinal tract does not allow for spore germination. In this type of botulism, there is no actual infection with C. botulinum.
Physiology/Pathophysiology
- 1.
Regardless of the means of acquisition, weakness and paralysis result when the toxin irreversibly binds with presynaptic peripheral cholinergic nerve endings (i.e., neuromuscular junction and autonomic ganglia), preventing stimulation-induced acetylcholine release by the presynaptic nerve [2, 3].
Nerve ending at neuromuscular junction. US National Institutes of Health, National Institute on Aging created original. Marked as public domain. (Published with kind permission of © Colin G. Kaide 2019. All Rights Reserved)
Published with kind permission of © Colin G. Kaide 2019. All Rights Reserved
- 2.
Recovery only results when new nerve terminals sprout and form new synaptic contacts in a process that may take months.
- 3.
Outbreaks of botulism have been described with ingestion of contaminated food, usually secondary to poor food preparation including home canning with an associated anaerobic, low acid, and low solute environment [4].
Of note, a major outbreak of foodborne botulism was noted in 2015 associated with a church potluck meal in Lancaster, Ohio, causing one of the largest outbreaks in United States (US) history with 21 confirmed cases, resulting in one death. The culprit was potato salad made from home canned potatoes [4].
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