A Berkwitt et al. NEJM 2024; 390: 358-366.Case 3-2024: An 8-Week-Old Male Infant with Inconsolable Crying and Weakness

This case report describes an 8 week old who been well until 7 days before the current presentation, when irritability, less frequent stooling and frequent crying developed. He was seen by his primary care clinic and symptoms were attributed to gas. Several days later, he presented to the emergency department with persistent crying but he had developed lethargy and weakness.

This case report details potential reasons for irritability in infants:

• Infections
• Neurologic causes including hydrocephalus
• Ocular/skin such as a corneal abrasion and hair tourniquet
• Cardiopulmonary causes such as heart failure and myocarditis
• Gastrointestinal causes like colic, constipation gas, and reflux
• Genitourinary like hernia and torsion
• Musculoskeletal like fractures
• Cancer including neuroblastoma and leukemia
• Metabolic causes
• Ingestions/Toxins

Then, the authors turn their attention to potential reasons for hypotonia:

• CNS disease -accounts for 60 to 80% of cases of hypotonia, specifically hypoxic–ischemic encephalopathy and cerebral palsy
• PNS disease -need to be considered in those with normal neuroimaging. To have an acquired PNS disease, the authors considered mainly botulism and Guillain-Barre syndrome.

Ultimately, the authors concluded that the infant likely had botulism which was in fact the correct diagnosis, confirmed by a stool test for Clostridium botulinum toxin type A (generally available only through local public health departments). Also, “On further interviewing, the patient’s family members reported that he typically had hard stools every 2 to 3 days. Two days before admission, the infant appeared to have abdominal discomfort, which his family members presumed was from constipation or gas. Honey was given to try to soothe him.”

Teaching points:

1. Don’t give honey or corn syrup to a baby, though “clearly defined food exposures, such as exposures to honey or corn syrup, account for only a minority of cases.⁸..Often, there is a history involving rural living, dust production, or nearby soil perturbation.⁹“
2. Give Baby BIG (infant botulism immune globulin) while waiting for results.
3. Try to ascertain dietary exposures when obtaining a history.

Fortunately, this infant fully recovered.

Related blog post: Why call it botulinum?