Hot Seat # 71: 17 yo F w/ subacute neurologic changes in setting of recent IVIG infusion
Posted on: March 14, 2016, by : Jeremy Root
Jeremy Root MD, Children’s National Health System
With Sabrina Guse MD, Children’s National Health System
The Case
17 year-old female with a history of POTS and Ehlers-Danlos syndrome presents to the ED with headache, lower back pain, weakness, and inability to talk for one week. Mother says patient was seen by her cardiologist three weeks prior to visit and was recommended to start IVIG due to decreased ejection fraction and severe POTS. Infusion occurred one week ago during which time she developed headache and lower back pain. Since treatment, she has had persistent headache and lower back pain, and has also developed increasing weakness, left side greater than right side. She has been unable to brush her teeth or hair and is unable to talk. She called her pediatrician who told her to come to hospital. This is her first presentation to your hospital system.
ROS: Denies fevers, URI symptoms, vomiting, diarrhea. Denies bowel or bladder incontinence.
PMH: POTS, Ehlers-Danlos, GERD, asthma, hx of possible brain aneurysm on brain MRI last year
Home meds: propranolol, florinef, mestinon, desmopressin, xyzal, escitalopram, Zofran, vyvanse, levocarnitine, phenergan, prevacid, dulera, tramadol. Recently completed week of decadron after reaction to IVIG.
PSH: multiple endoscopies w/ biopsies, multiple bronchoscopies, s/p PICC placement with associated thrombus
Social Hx: previously a swimmer and track athlete, lives in West Virginia
Exam: T36.4, HR 92 RR 16, BP 115/72, 100% on RA
Gen: Tired appearing, not able to talk, uses cell phone to communicate (types and phone reads aloud)
CV: RRR, no murmurs, 2+ pulses, cap refill < 2 secs
Resp: clear lungs, non-labored respirations
GI: surgical scars evident, soft, NT, no organomegaly
Neuro: nonverbal, communicates by hand gestures, nodding and iPhone; EOMI, PERRL, able to close eyes and smile without deficits, face symmetric; tongue with + fasciculations, facial sensation intact, hearing intact, able to lift bilateral arms and legs against gravity, 3/5 strength in upper and lower extremities, 3+ patellar and Achilles reflexes, negative Babinski, normal peripheral sensation
You obtain basic labs and give a NS bolus.
Question for you:
Labs:
WBC 8.1, Hemoglobin 12.4 Hct 37.3 Platelets 149
Na 139 K 3.9 Cl 104 CO2 26 BUN 19 Cr 0.53 Glu 72 Ca 8.3
UA: 1+ protein, negative for WBC, LE, nitrites, neg ketones, HcG neg
Another question:
You consult transfusion medicine who says POTS patients can have atypical reactions to IVIG, although this would be very atypical.
One last question:
How would you approach this case? Please share your opinions by clicking on “What do you think?” below.
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You could spend 3 hours on this case, or 5 minutes….depends how busy your ED is, how much you trust your strength examination, how much you believe any of the patient complaints, and how likely you will be to discharge the patient or solve the dilemma in less that 5 notes. This would be a good editorial on Emergency Medicine Opportunity Cost.
I would’ve added a CK, CRP, and LFTs to her lab eval to look for toxicities to any of her 300 medicines. Need to get a better strength exam (stand her up) and delve into her aphasia a bit more….I’m having trouble tying a vagus-laryngeal nerve problem with decreased strength. Very different parts of the CNS here and I’d have a hard time believing this is all cortical.
These cases aren’t difficult at tertiary care centers when neurologists will come into the ED and every specialist is at your disposal….but how would you approach this patient at Anne Arundel for example where you can get an MRI in the ER….and the MRI/MRA comes back completely normal?
This is confusing because of the variety of neurological concerns. To group them in a way that made sense to me:
– Back pain and HA: aseptic meningitis could explain these symptoms, in the context of an IVIG infusion at the outset of the symptoms. Infection: no evidence of meningitis on exam. Trauma: no history, though people with Ehlers-Danlos can have spontaneous bruising from fragility of the vessels (though not sure if dural bleeds can occur spontaneously).
– Limb weakness: Is this global weakness- drug effect, metabolic derangement (Mg, Na, Ca), anemia, infection, stroke, demyelinating disorders, neuromuscular disorder (always botulism, other), depression. Is this more confined to large muscle groups, as in a myositis from a viral illness, from medications. Need to check the exam to know if the weakness is confined to the proximal muscles, as occurs in polymyositis.
– Tongue fasciulations: this is either the direct effect of medications or an upper motor neuron sign, as in a stroke.
– Difficulty with speaking: she can type her thoughts on her phone, so she is clearly not confused. But I am not clear whether she is too weak to produce the sound (in which case I would expect a quiet or broken up conversation) or whether she has trouble producing the correct words (expressive aphasia)—the latter suggests a central process of infection or stroke, while the former is more peripherally focused, such as mentioned above.
I am not clear on how these fit together in one narrative. The asymmetric limb weakness is more concerning for a stroke, given the presence of tongue fasciculations. At the same time, she is on a lot of medicines that have potential to interact or act directly. And never discount the power of depression.
For this situation, I would have a conversation with the Neurologist to help focus on an anatomic location for the symptoms before imaging. Add an aldolase to the CK—this is a good screen for muscular dystrophies.
And look at her quality of breathing. If there is any concern of increasing weakness, I can place her on EtCO2 and ask the RT to measure a NIF.
Am hard pressed to do anything further here but since the patient and family is from some distance it seems a bit hard to discharge with the “return of the symptoms get worse” mantra, the slew of medications and the underlying diagnosis.
The symptoms and physical findings are not congruent, but there are a few things we have not ruled out, like ADEM. Depending on day of week and time of day, I’d vote for an observation admission and MRI.
This case is interesting and challenging, because the differential diagnosis is so broad. There are many things going on with this patient, including the host of medications she is on. The differential has already been well outlined by Jennifer Chapman above: infectious, neurologic, rheumatologic, oncologic, medication side-effect. As per my usual approach, I would want to address the potential “big bad things” with a thorough work-up, while acknowledging that I have seen patients present like this with conversion disorder, especially when they present with an array of neurologic complaints that cannot be tied together.
The 1st step would be a more thorough exam. In particular, we need to attempt to characterize the weakness further. Proximal vs. distal? Gait? Also, is there any back TTP? Is she truly unable to speak?
I would expand the labs to include ESR/CRP, CK, LFTs and aldolase. I would pursue neuroimaging. However, given that the patient is stable and symptoms have been present for a week without clear worsening, it would be reasonable to consult Neurology, if available, prior to obtaining imaging. I am impressed by her significant leukocytosis. Could she have an indolent spine process despite lack of fever that could explain her back pain and lower extremity weakness? Discitis is less common in adolescents, but can occur and patients are typically afebrile. Vertebral osteo? Given her appearance, less likely an epidural abscess. These still wouldn’t explain all her symptoms. I would be inclined to get both brain and spine imaging, but this would be one of the questions to further address with Neurology. And finally, as Jennifer mentioned above, if I have any concern regarding progressive weakness, a NIF needs to be checked.