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MORNING REPORTD Spencer Mangum, PGY38/12/13

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Patient

While cross covering on Lahey on 5/6/13, you meet:

7 yo female with relapsed preB ALL s/p transplant, with a2nd relapse after transplant. Admitted one month prior for an experimental Phase I study (to

stay through count recovery), that includes the following: Decitabine, Vorinostat, Mitoxantrone, Dexamethasone, Vincristine,

PEG-asparaginase and IT methotrexate

In the afternoon, the nurse calls you regardingbradycardia (had been occuring previously). Electrolytes

were checked (wnl) as well as an EKG (wnl). While getting a blood transfusion that evening, noted to

have up-trending blood pressures (systolic 120's)

At ~10pm after finishing the pRBCs, the pt acutelydeveloped HA, Nausea / vomiting (NB/NB), confusion,and a BP of 150/100's with continued bradycardia (50's).

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Patient

Her HA / confusion resolved and BP significantly improved(systolic 130's) within minutes. Neuro exam was wnl (Awake /alert / responsive, PERRL, CN 2-12 intact, no focal deficits).Although continued nausea / vomiting.

Given rapid improvement, it was elected to continue to watchthe patient closely with q1 hrBPs and neuro checks. (Neuroexam remained reassuring, by midnight BP was 110/64).

At approximately 4-4:30 AM, the nurse walked into the roomand found the pt. laying in the bed unresponsive and a codeblue was called. Pt had a pulse but minimal respiratory effort. Still unresponsive.

Systolic BP ~120 and HR in the 150's, Sinus rhythm on the monitor

Unsure if there was initial twitching.

Pt lost control of her bladder (ie, involuntary enuresis)

Pupils were dilated b/l but reactive.

Vital Signs and exam otherwise unremarkable

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Differential Diagnosis?

7 yo f with relapsed preB-ALL with a prior episode of HA,

Confusion, N/V and acute hypertension (improved), now

unresponsive and apneic.

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(Secret Slide)

When you check the computer for most recent labs:

CBC (that AM) WBC 0, Hgb 7.8, Hct 23.0, Plt 66

BMP (that afternoon): Na 141, K 3.7, Cl 108, Bicarb 21,BUN 12, Cr 0.22, Glucose 122

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(Top) Differential Diagnosis

Intracranial Hemorrhage

Should have increased concerns for this particularly if platelets are

less than 10 - 20

Sinus venous thrombosis

Acute Hyponatremia (causing cerebral edema / seizure)

Most commonly 2/2 SIADH - risk with certain chemo meds,

paraneoplastic, or other (lung / brain involvement)

Nausea / Vomiting and IV fluids

Ischemic Stroke

Hemorrhagic Stroke

Encephalopathy (toxic or metabolic 2/2 new chemo?)

Seizure NOS

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How would you manage the code?

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How would you manage the code?

Pt bag masked given poor / absent respiratory

effort

CBG / iStat electrolytes obtained

pH 7.02, pCO2 113, HCO3 28.9, Base Deficit 5

Na 141, K 4.3, Glucose 142, iCa 1.25

Given Ativan 0.1 mg/kg x2

Pt was intubated and taken for a stat head CT

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Patient (continued)

Head CT: wnl

Head MRI: Increased T2 signal in the cerebellar hemispheres, occipital lobes,

and, to a lesser degree, frontal lobe white matter. No cytotoxic

edema. Most consistent with PRES.Admitted to the PICU intubated. Given a Nicardipine gtt

x2 hours and started on Lasix 10mg BID and Amlodipine5mg PO daily for HTN. Dexamethasone was held.

Neuro consulted: PRES (posterior reversible

encephalopathy syndrome) is the most consistentdiagnosis given her MRI findings and her episodes ofhypertension in the setting of dexamethasone.

Extubated, returned to baseline mental status, and

transferred to the floor within 1-2 days.

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PRES (Posterior Reversible

Encephalopathy Syndrome) Officially now known as Reversible posterior

leukoencephalopathy syndrome (RPLS)

Radiographic syndrome, likely heterogeneous etiologies thatare grouped together because of similar findings onneuroimaging studies.

Symptoms: Seizures

Can be tonic clonic. Often the presenting manifestation

Headaches Constant, nonlocalized, moderate to severe, and unresponsive to analgesia

Altered consciousness Mild somnolence to confusion and agitation progressing to stupor or coma

Visual disturbances

Imaging findings: Symmetrical white matter edema in the posterior cerebral

hemispheres, particularly the parieto-occipital regions

Seen on MRI, NOT CT

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PRES (Posterior Reversible

Encephalopathy Syndrome) No specific diagnostic criteria

Diagnosis made when symptoms present with consistent

neuroimaging.

Most often develops in association with:

Hypertension

PRES: elevation of blood pressure beyond the upper limits of cerebral

autoregulation, leading to cerebral hyperperfusion and vasogenic

edema

Rapidly developing, fluctuating, or intermittent hypertension carries a

particular risk for hypertensive encephalopathy With chronic hypertension, cerebral autoregulation is able to adjust.

Immunosuppressive therapy

Mechanism still poorly understood. (Cytoxic mediated endothelial cell

dysfunction?). Most commonly citied medication is cyclosporine,

although tacrolimus / sirolimus and bevacizumab have also beenimplicated.

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PRES (Posterior Reversible

Encephalopathy Syndrome) Treatment:

Focuses around normalizing the blood pressure

For patients with seizures, long term Antiepileptics not needed(none were continued for this patient)

In some case series, patients given AEDs (phenytoin) for one to threemonths after

In rarer cases, recurrent seizures can occur several months after anPRES episode and these patients are maintained onAEDs

Prognosis: With prompt recognition and removal of the inciting factor / good

blood pressure control: Is fully reversible in most cases over days to weeks

Radiologic improvement lags behind clinical recovery.

When unrecognized:

Continued cerebral hyperperfusion leads to further vasogenic edema

and can progress to ischemia, massive infarction, and death.

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Patient Summary with PRES

7 yo female with several risk factors: Immunosuppression with chemo (although not cyclosporine)

Acute episode of hypertension

Secondary to dexamethasone?

Contribution of pRBC transfusion via increased fluid load?

Initial Headache, Nausea / Vomiting, AMS were likely firstsymptoms of PRES Likely had acutely increased ICP / cerebral hyperperfusion with this

episode

Given rapid improvement, not worked up further until patient had a

(likely) seizure Diagnosis made with MRI! (NOT CT)

Patient returned to baseline relatively quickly with aggressiveBlood Pressure management Transitioned to oral anti-hypertensive meds for a few weeks until no

longer getting dexamethasone. No further AEDs after initial episode.