A diagnosis of adult-onset “infant” botulism was confirmed by identification of botulinum toxins A and E in stool and serum of a 32-year-old woman with cystic fibrosis treated for a pseudomonas pneumonia and having a percutaneous gastrostomy tube placed for poor intake and weight loss, at the Department of Neurology, University of Minnesota, Minneapolis. Wound infection was not established, but 2 weeks after gastrostomy the patient developed nausea and vomiting, with ileus, progressive dysphonia, neck and proximal weakness, and respiratory failure. Partial ptosis and slowly reacting pupils were noted 2 days later, but extraocular movements, tendon reflexes, and sensation were normal. EMG studies were suggestive of presynaptic neuromuscular junction defect. Motor unit potentials were of small amplitude and brief duration with increased polyphasia. There was early recruitment with interference pattern and reduced peak-to-peak amplitude. Increments in CMAP amplitude with 50-Hz stimulation, and single-fiber EMG of extensor digitorum were abnormal. Sensory and motor conductions in all limbs were normal. Stool culture grew Clostridium botulinum, but the source of the infection was not identified. [1]
COMMENT. Adult-onset infantile botulism is rare, but this case report is of interest because the patient was suffering from cystic fibrosis, a childhood disease, which affects intestinal motility and, along with prolonged antibiotic therapy, predisposes to colonization of the gut by C botulinum. The diagnosis should be considered in a child admitted to the intensive care unit in respiratory distress, and presenting with nausea, vomiting, and fever that precede the onset of ptosis, nonreactive pupils, dysphagia, dysphonia, weakness of facial and neck muscles, followed by flaccid paralysis. Sluggish pupils and paralytic ileus were clinical clues in the differential diagnosis.