What is wrong with these kids? (Mystery Diagnosis)

Specialties PICU

Published

My area has recently seen 3 children at 2 separate facilities present with eerily similar and so far undiagnosed problems. Similar age group, but nothing else in common (different locations, ethnicities, no similar contacts or travels, etc.)

Presentation: 2-3 day history of fever, c/o headache, occasional vomiting. ER admission due to changes in level of consciousness or physical mobility.

Work-up: CT, MRI, LP, pan-cultures, viral panel of CSF, blood and secretions, standard lab work-up. All repeated after a few days of admission. Regular blood cultures. All results unremarkable. WBC's normal, CRP mildly elevated in 1-2 patients, CT and MRI normal, spinal fluid clear, cell count, protein, glucose results normal. All cultures and panels persistently negative.

Treatment: Broad-spectrum antibiotics and antivirals including max doses of vancomycin, ampicillin and acyclovir.

Course of stay: Progressive decline in neurological status. Most recent presenting patient deteriorated from oriented and physically appropriate to a GSC score of 6 within 8 hours. Seizures in the first 2 presenting patients. Loss of ability to maintain airway. Intubated for currently 2 weeks and 4 weeks respectively.

We are all at a loss. Does anyone have any insight?

Look at what they have been eating. May be due to pesticides/herbicides on fresh foods.

Also check whether they have had immunizations recently.

Specializes in PICU, Sedation/Radiology, PACU.

No PMH. All of these patients were healthy previous to their admission. Immunizations UTD on most, with no recent vaccinations in the past 6 months. CSF and blood was sent for a viral panel- I'm not sure if West Nile is included in these results but last time I checked the full results of the viral panel were still pending for the one patient left on our unit (the other was transferred at the request of the understandably frustrated parents searching for answers).

The operating assumption is unspecified viral encephalitis. And actually, the patient on our unit is improving slowly and was actually extubated recently. The other three, to my knowledge, remain intubated. I suppose it's possible that we may never know exactly what caused this, which would be a rather anti-climactic end to this thread.

Because of the similar symptoms, it seems like their has to be something in common, but so far nothing has been noted. Different locations, different schools, never met before, no similar contacts that we know about, no school trips, and we live in a fairly urban area so there is limited contact with exotic animals. Pets are an option, but in four separate households it just seems strange. Something environment you'd expect a more wide-spread outbreak.

I'm in NJ and I'd rather not specify any more than that. To my knowledge the CDC is not involved yet, although there has been talk of bringing it to their attention.

Specializes in NICU, ICU, PICU, Academia.

Thanks for the update. I think about this thread often. It's just too coincidental with the demographics, don't you think?

Specializes in Adult Internal Medicine.
No PMH. All of these patients were healthy previous to their admission. Immunizations UTD on most, with no recent vaccinations in the past 6 months. CSF and blood was sent for a viral panel- I'm not sure if West Nile is included in these results but last time I checked the full results of the viral panel were still pending for the one patient left on our unit (the other was transferred at the request of the understandably frustrated parents searching for answers).

The operating assumption is unspecified viral encephalitis. And actually, the patient on our unit is improving slowly and was actually extubated recently. The other three, to my knowledge, remain intubated. I suppose it's possible that we may never know exactly what caused this, which would be a rather anti-climactic end to this thread.

Because of the similar symptoms, it seems like their has to be something in common, but so far nothing has been noted. Different locations, different schools, never met before, no similar contacts that we know about, no school trips, and we live in a fairly urban area so there is limited contact with exotic animals. Pets are an option, but in four separate households it just seems strange. Something environment you'd expect a more wide-spread outbreak.

I'm in NJ and I'd rather not specify any more than that. To my knowledge the CDC is not involved yet, although there has been talk of bringing it to their attention.

I have ready over this many times, and I am always happy to see updates. It has become about of a diagnostic challenge for me and I oft consider what my impression would be if I was the provider (though I haven't seen kids in a while).

Viral encephalitis would have been my primary working dx, given the presentations.

I would consider the zebras:

Murine typhus, ADEM, RMSF, Q fever.

Specializes in Pediatric Pulmonology and Allergy.

It's also possible that these cases are unrelated and any similarities are coincidental.

I found this helpful article on viral encephalitis.

Viral encephalitis - New York Methodist Hospital

Specializes in Medsurg/ICU, Mental Health, Home Health.

I am just a state away and our region tends to have the earliest West Nile cases every year, it seems.

Q-fever - one of my favorite zebras...as an aside. :)

Specializes in PICU, NICU, L&D, Public Health, Hospice.

Any update?

We have seen multiple pts with teratomas, resulting in severe neuro symptoms from constant movement, to seizures. The ages varied from toddler to teenagers, both male and female.

do not assume or relate the symptoms to a definite medical condition, this will just isolate one of the symptoms that the patient is displaying, but instead, run tests that is applicable to the patient's symptoms and conditions to determine the cure that must be applied onto the patient...

Specializes in NICU, ICU, PICU, Academia.

^^^^ what? ^^^^

Specializes in PICU.

Check into primary amebic meningoencephalitis (PAM), particularly Naegleria Fowleri. Over a year ago, we had a case with similar presentation as you described and usual work up showing nothing. It turned out to be PAM.

Specializes in PICU, Sedation/Radiology, PACU.

Sorry that I haven't updated sooner.

There hasn't been any official diagnosis. However, I'm happy to report that the patient who was on our unit (actually the most recent patient to present with these symptoms, was able to be extubated after about two weeks. Other than some rapidly improving physical deficits, he appears completely intact neurologically- responding appropriately to questions and recognizing family members as soon as he was awake enough. I don't have an update about the other cases, since they aren't at our hospital anymore, but it's looking more and more likely that we may never actually find out what caused this in the first place.

Thanks for your thoughts and your input, as always.

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