The patient was a 6 – month – old male who presented with a 3 – day history of i

Question

The patient was a 6 – month – old male who presented with a 3 – day history of increasing hypotonia and a 1 – day history of dehydration. Three days prior to admission the mother had noticed decreased suck while breast – feeding. The next day it took him 1 hour to take his normal amount of breast milk. He normally took only 15 minutes. During the evening 2 days prior to admission, the parents noticed that the infant had generalized weakness with decreased movement and difficulty sitting up. That evening the child would not breast – feed. The morning prior to admission the parents noted that the infant was increasingly floppy and took only 1 oz of breast milk (versus his usual 4 1/2 oz.) and 1 oz. of juice. He had trouble with gurgling in the back of his throat, very poor head control, and increased floppiness. They took him to a local physician, who noted severe hypotonia and dehydration. The parents were told to take their son to the local emergency room. On review of systems, it was noted that the child began to be constipated 4 weeks previously and had had only two small stools over the last 6 days. His diet consisted of breast milk, occasional sweet potatoes, and rice cereal. Abnormal findings on physical examination consisted of generalized hypotonia with head lag. Cerebrospinal fluid findings were within normal limits. The patient was admitted to the pediatric intensive care unit. Because of increasing respiratory difficulty, he was intubated. He remained on the ventilator for 6 days, was extubated, and was discharged home 11 days after admission. A stool specimen obtained at admission was positive in a mouse lethality test.

1. What condition did this child have? What clinical clues are present that help you make this diagnosis?

2. What organism caused this condition? Describe the epidemiology and pathogenesis of this disease.

3. How is this form of the disease typically treated?

4. What other forms of disease can be seen with this organism? Describe the similarities and differences in these different forms of this disease.

5. The agent causing this child’s illness has been used therapeutically. How is it used, and for what conditions is it used? What risks have been associated with its therapeutic use?

Explanation / Answer

1. The child is suffering from botulism. Hypotonia, dehydration, dysphagia(difficulty in feeding), loss of neck holding, respiratory(due to weakness of respiratory muscles), and positive mouse lethality test with normal CSF findings are clinical clues leading to botulism.

2. It is caused by Clostridium botulinum. Clostridium botulism spores are uubiquitous, In USA, 60% of the cases found east of the Mississippi river are due to B toxin, while west of the river, 85% of cases are due to toxin A. Infant botulism is commonest form, occurs with toxin types A, B, or F. About 80 to 100 pediatric cases of botulism are reported every year. A total of 2419 infant cases were reported in the US from the year 1976 to 2006. Over this time span, an additional 524 cases of infant botulism were identified globally. Rare cases of infant botulism due to toxin E production by Clostridium butyricum, or toxin F production by Clostridium baratii, have also been reported.

Pathogenesis: botulinum toxin acts by blocking the production or release of acetylcholine at the synapses and neuromuscular junction. It causes a symmetric descending paralysis in the characteristic pattern ending in death by respiratory paralysis.

3. Management includes supportive care and assisted feeding.

Antibiotics and antitoxins are not indicated.

Ventilatory is usually given for the cases of respiratory failure.

4. Other forms of disease are food poisoning and wound botulism.

Common symptoms: ocular paresis, difficulty in swallowing, death due respiratory failure.

Differences: Vomiting, thirst is usually present in present in foodborne botulism, however, it is absent in wound botulism and infant botulism.

5. Botulinum toxin is used for treatment of hyperhidrosis(excessive sweating), overactive bladder, migraine, neuralgia, etc.

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