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Abstract Details

Acute Descending Paralysis and Respiratory Distress: Myasthenia Gravis vs Wound Botulism
Child Neurology and Developmental Neurology
Child Neurology and Developmental Neurology Posters (7:00 AM-5:00 PM)
075

Objective: Recognizing traumatic wound botulism as a cause of flaccid paralysis and respiratory distress. 


Background: Pediatric botulism is a serious paralytic illness caused by a toxin produced by the bacterium Clostridium botulinum. Foodborne botulism can be caused by eating foods containing botulinum toxin. Infant botulism occurs when ingested C. botulinum spores colonize and produce toxins in the intestines of the affected infants. The least common form of botulism is wound botulism in which C. botulinum bacteria produces toxin in a contaminated wound.


Case Presentation: A 14-year-old female began experiencing acute progressive ocular involvement with ptosis and esotropia, bulbar weakness leading to respiratory failure, and upper extremity weakness after forearm open fracture and surgical repair complicated by wound infection. Initial differential diagnosis included Guillain-Barre syndrome, myasthenia gravis, botulism, and other toxin-mediated diseases. Median nerve motor response was mildly reduced which improved after short exercise.  Low frequency repetitive nerve stimulation (RNS) in a clinically weak APB muscle showed decremental response while EMG showed non-irritable myopathic changes. High frequency RNS of the right median nerve did not demonstrate an incremental response. These findings heightened suspicion of myasthenia gravis onset in a crisis. However, because these electrodiagnostic findings could not distinctly rule out presynaptic NMJ disorder, the alternative diagnosis of dirt-contaminated botulism wound infection remained possible. Wound cultures and serum toxin assay later demonstrated active botulism. To ensure broad treatment coverage, she was treated with the heptavalent botulinum antitoxin as well as intravenous immunoglobulins and improved, eventually being discharged 77 days after hospital presentation.


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Conclusions: Botulism is a serious disease in pediatric patients presenting with progressive flaccid paralysis. Electrodiagnostic studies remain a cornerstone test in diagnosis of NMJ disorders, but its specificity can be insufficient to differentiate pre-synaptic from post-synaptic disorders. Therefore, early diagnosis and treatment of botulism depends on comprehensive clinical evaluation.


Authors/Disclosures
Adam C. Kneepkens
PRESENTER
Mr. Kneepkens has nothing to disclose.
No disclosure on file
Peter Karachunski, MD (University of Minnesota) Dr. Karachunski has nothing to disclose.
Sonya G. Wang, MD (University of Minnesota Department of Pediatrics) Dr. Wang has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ovid Therapeutics.