Welcome to PICU Doc On Call, A Podcast Dedicated to Current and Aspiring Intensivists.
I'm Pradip Kamat and I'm Rahul Damania. We are coming to you from Children's Healthcare of Atlanta - Emory University School of Medicine.
Welcome to our Episode an 8-year-old admitted for PRESS syndrome with altered mental status secondary to seizures.
Here's the case presented by Rahul:
Our patient today is an eight-year-old who was admitted to the floor with a diagnosis of MIS-C. On his initial echo, his EF had mildly depressed systolic function, dilatation of coronaries, and worsening of inflammatory markers. As a result, the care team increased the dosing of the methylprednisolone administered to this patient. Since the initiation of methylprednisone, The patient's SBP had been steadily increasing with the latest systolic values approaching 140s-150s.
On hospital day 3 patient had a generalized tonic-clonic seizure and became unresponsive for which a rapid response on the floor was called. The patient was emergently bagged and brought to the PICU for airway protection and intubation
Initial vitals on PICU admission: He was afebrile, mildly tachycardic, and hypertensive to 160s even after sedation.
In the PICU an initial head CT scan done after intubation and stabilization of the patient showed no bleeding or mass. cEEG monitoring was initiated, neurology consulted and an MRI was ordered for the following day. As his AMS was thought to be related to his BP, the team pursued BP control with Nicardipine.
To summarize key elements from this case, this patient has:
Absolutely, the differential is broad, however, right now I am thinking of an acute stroke categorized as hemorrhagic, ischemic, or venous thrombotic; a meningoencephalitis, CNS vasculitis, acute demyelinating encephalomyelitis, metabolic encephalopathy, tumor, or AMS related to hypertension.
Pradip, let's transition into some history and physical exam components of this case?
What are key history features in this child?
Rahul, are there some red-flag symptoms or physical exam components which you could highlight?
To continue with our case, Rahul ,what were the patient’s labs were consistent with:
OK to summarize, we have:
An eight-year-old, with acute severe hypertension, seizure altered mental status, and MRI changes suggestive of vasogenic edema in the posterior part of the brain -all this brings up the concern for posterior reversible encephalopathy syndrome (PRES) the topic of our discussion today.
Rahul ,Let's start with a short multiple-choice question:
A 19-year-old with h/o of renal transplant on tacrolimus and recent initiation of steroids for rejection presents with acute severe hypertension and a GTC seizure. The patient is afebrile with no rash. CT scan at OSH reveals no mass or hemorrhage. After stabilization and initiation of antihypertensive therapy, the next study of choice for diagnosis is
Rahul, the correct answer is B) MRI. Patients such as the one described in the above question are at high risk to develop PRES. MRI will show classic changes associated with PRES- Involvement of the parieto-occipital region of the brain. Vasogenic edema (typically affecting the brain white matter) is characterized by hyperintensity on FLAIR and T2-weighted MRI sequences. As seizure is a presentation of PRES as in our case above, cEEG monitoring especially if intubated is indicated but may not be helpful in diagnosis. An LP also will not help with the diagnosis of PRES and the patient in this question is afebrile. PET scan may have a role in unusual or atypical cases of PRES mainly to distinguish it from the tumor. There is decreased fluorodeoxyglucose (FDG) and Methionine(MET) uptake in most PRES cases compared to tumors such as gliomas or lymphomas.
The diagnosis of PRES relies on a combination of clinical presentation and neuroimaging. Acute or subacute presentation with encephalopathy, generalized tonic-clonic seizures (60-75% patients), headaches, visual field deficits, cortical blindness, hallucinations, or rarely focal findings such as aphasia or hemiparesis should raise suspicion for PRES. Headache+visual disturbances+generalized tonic-clonic seizures =PRES unless proven otherwise.
Rahul, as you think about our case, what would be your differential?
Rahul, can you comment on the pathogenesis of PRES**
It is hypothesized that when the patient’s mean arterial BP exceeds the upper limits of cerebral autoregulation it leads to hyper-perfusion and the breakdown of the blood-brain barrier allowing interstitial extravasation of plasma and macromolecules and subsequently vasogenic edema.
Pradip, which patients are at risk for PRES?
Apart from acute severe hypertension, a number of other conditions are associated with PRES. PRES is seen in patients receiving immune suppression, especially calcineurin inhibitors (tacrolimus or cyclosporine) after a stem cell or solid organ transplantation. Higher incidence is seen in BM or stem cell transplant as the dose of immunosuppression is higher in these patients compared to solid organ transplant patients. Autoimmune disorders, pregnancy with pre-eclampsia, eclampsia as well as those with renal disease have been linked to PRES.
Here's a summary point when you see progressive hypertension in a patient post-transplant, after doing due diligence to pain control and diagnostic workup, pay close attention to mental status as these patients (especially if they are immunosuppression) are at high risk for developing press.
Pradip If you had to work up this patient, what would be your diagnostic approach?
The presence of vasogenic edema affecting white matter in the parieto-occipital regions of both hemispheres (rarely asymmetric) on MRI FLAIR in the appropriate clinical context is highly sensitive for PRES. Neuroimaging helps to exclude alternative diagnoses such as brain tumor or acute demyelinating syndromes as well as detection of intracranial hemorrhage.
Rahul, if our history, physical, and diagnostic investigation led us to PRES as our diagnosis what would be your general management of framework?
This concludes our episode on posterior reversible encephalopathy (PRES) We hope you found value in our short, case-based podcast. We welcome you to share your feedback, subscribe & place a review on our podcast! Please visit our website picudoconcall.org which showcases our episodes as well as our Doc on Call management cards. PICU Doc on Call is hosted by me Pradip Kamat and my co-host Dr. Rahul Damania. Stay tuned for our next episode! Thank you!
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