Pediatric Pearls – Floppy Baby
Ilene Claudius MD and Sol Behar MD
Take Home Points
- Botulism is a clinical diagnosis and early initiation of appropriate treatment may result in better outcomes.
- Fatigable pupils are associated with botulism.
- Construction, earthquakes and honey containing products or home remedies have been associated with botulism.
- Constipation is often the initial presenting symptom of infantile botulism.
- Behar was working with one of his chief residents. They saw a seven month old baby brought in by the mother. The mother reported that although the baby had been able to sit up by himself previously, he was no longer able to do so. She opened her purse and it was filled with homeopathic remedies. She wasn’t very concerned. She reported initially the baby would fall to the side, but now he just didn’t have the tone to keep himself up.
- The baby did not have any fever, cough or recent illness. No vomiting or diarrhea. He hadn’t pooped for a few days which was normal for him (and one of the reasons she put him on probiotics). He choked when he was drinking milk.
- The baby was in the mom’s arms. He was like a rag doll sliding through her arms. By 7 months, he should have enough tone to cling to the mother like a koala bear. When he laid down, he was like a starfish with his arms and legs extended. When lifted by his arms, his head lagged behind. He was globally hypotonic.
- Alternative or complementary medications aren’t tested or regulated in the way actual medications are. They don’t have to be shown to work or to be safe. They don’t even have to contain the ingredients listed on the label. Claudius does not recommend the use of these products in children this age.
- The child was afebrile. His vital signs were normal. His heart and lungs were unremarkable. He looked like Garfield the cat. He had a profound ptosis. Both eyelids were at half-mast. He was globally floppy and vocalizing. He was attentive but looked sleepy and hypotonic.
- What is your differential diagnosis?
- Acute flaccid paralysis most commonly affects one or two limbs.
- Congenital conditions such as spinal muscular atrophy usually present earlier although there are different types. This is characterized by absence of reflexes.
- Claudius has seen some weird cases of myasthenia gravis in children.
- The child was given supplements and you need to consider botulism.
- What did they do? Behar called the neurologist. If you do think it is infantile botulism, it is a clinical diagnosis and the earlier you give the baby the immunoglobulin, the better the outcome may be. When Behar shined the light in the child’s eyes, they constricted normally. When he held the light there, the pupils began to dilate. Fatigable pupils are specific for botulism.
- What is the botulism toxin and how does it work? The toxin causes a functional denervation of skeletal and smooth muscle. It happens at the synaptic level. It binds to the presynaptic membrane and prevents the release of acetylcholine. You can’t get neurotransmission and it results in global weakness. This manifests in smooth muscle as constipation. This is almost always the initial presenting sign of infantile botulism. This may be followed by descending weakness, global hypotonia and cranial nerve abnormalities such as ptosis. They may develop automatic instability, diaphragmatic paralysis and respiratory failure.
- Why does the treatment differ in infants? Babies don’t have the stomach acid or motility to prevent the spore from forming toxin. If you can get the immunoglobulin on board, you can prevent complications and decrease the duration of illness.
- What happens if you don’t give BabyBIG (botulism immunoglobulin)? This is expensive at about $45,000 per dose. You may need neurology approval to order it. If you let this go, the typical duration of disease is about 2 months. Occasionally, there may be relapses. These children will likely require ICU and tube feeds. If treatment is initiated early, you may have a good outcome.
- What are causes infantile botulism? Being near a construction site or earthquake where spores are aerosolized. Giving honey. Some of the cough and cold remedies have honey in it. Honey is only responsible for about 15% of cases but is a more easily modified risk factor.
- There is a new pacifier on the market that contains honey.
- Additional history was obtained when the patient was in the ICU. The family had been redoing their driveway. The illness was more likely due to spores kicked up from the construction than homeopathic remedies.
Sean G., M.D. - October 3, 2019 2:39 AM
I think u could avoid bashing naturopathy and homeopathy. Lets remember that our threshold is "non inferior to placebo" and no obvious harm suspected....and the absolute fraudulent behavior of Big Pharma and the complicity w Big Pharma of the supposed "watchdog" agencies. Remember stroking pts out w SL nifedipine in the 90's? How about all those "harmless" NSAIDS we pushed on people for decades before we figured out they can cause ACS? How about all the expensive immune agents marketed to people with psoriasis, and the 5 min car salesmen blurb at the end of the commercials pointing out sudden death has been reported....but hell their skin was clear! How about Abilifly being the number 1 drug sold for use in children w depression in 2015....I think 750 billion i sales? And its NEVER been studied in children!!!! Yeah I think the last thing we allopaths should be doing is laughing off herbal medicine because ours are "so good". Other than that, nice review.
ilene c. - October 3, 2019 12:10 PM
Good points. Not my goal to bash homeopathy. However, also think it is important to recognize how unregulated those medications are. Just like anything else, some aren't what they are advertised to be and some are. Doesn't mean that routine medications haven't been problematic as well. But I do suspect that when you give SL nifedipine, you are giving SL nifedipine. That doesn't make it safe or OK, it just makes it nifedipine. With many CAM therapies that patients purchase OTC, they may be getting something other than that which they intend to purchase. Both problematic, but not entirely the same set of problems.
Bradley K. - May 20, 2021 12:45 PM
Thanks Drs. Claudius and Behar for this case. Had a case of a 3 month old with poor tone. Constipation for 5 days and poor latch. Floppy on examination, but had dilatated fatigable pupils. Normal vitals. No known exposure to CAM, honey, or nearby construction sites. Thanks to this pearl we considered infantile botulism early and were able to transfer patient to tertiary care children's hospital early. We considered septic workup for baby with poor tone, but no hx of fevers. Deferred LP to children's hospital. Any thoughts on early administration of antibiotics worsening toxin release? Thanks for this case presentation to help clinch a diagnosis.
ilene c. - May 20, 2021 12:49 PM
Early administration of gentamycin is thought to worsen the paralysis. Anecdotally, I had a similar case many years back. We have amp and gent and by the morning, the child was a rag doll! I like to think of it as a diagnostic tool (totally kidding!). Great pick-up on your part!