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Posterior reversible encephalopathy syndrome (PRES) with intracerebral, intraventricular hemorrhage and cerebral vasculopathy

Case contributed by Dalia Ibrahim
Diagnosis almost certain

Presentation

Seizures, blindness and deterioration of mental status after cardiac surgery followed by sepsis and unstable vital signs.

Patient Data

Age: 20 years
Gender: Female

Bilateral fairly symmetrical frontoparietal and occipital subcortical white matter vasogenic edema eliciting high signal at T2 & FLAIR WI with no diffusion restriction.Those lesions are predominantly within the occipital lobes. Similar patches of abnormal signal are seen at the medial thalami, periaqueductal midbrain and cerebellar dentate nuclei.

Intra-ventricular hemorrhage is noted with mild hydrocpephalic changes.

SWI images shows multiple microhemorrhagic foci within the vasogenic edema.

MRA shows vessel irregularity of the anterior, middle and posterior cerebral arteries with multifocal segments of vasospasm, giving the "string-of-beads" appearance.

MRV appears normal.

1. Bilateral cerebral (predominantly occipital) vasogenic edema (yellow arrows)

2. Intraventricular hemorrhage (blue arrows in 2 & 3 images)

3. Microhemorrhagic foci within the vasogenic edema (red arrows)

4. MRA showed vasculopathy with multifocal stenotic segments of vasoconstriction (green arrows)

Case Discussion

The case demonstrates multiple typical features of PRES in the form of cerebral predominantly occipital and frontoparietal vasogenic edema associated with multiple parenchymal microhemorrahgic foci within the areas of vasogenic edema as well as intraventricular hemorrhage.

Also, the MRA features were typical for PRES induced vasculopathy evident by vessel irregularities of the ACA, MCA & PCA arteries secondary to multifocal segments of vasospasm giving the "string-of-beads" appearance.

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state that occurs secondary to the inability of the posterior circulation to autoregulate in response to acute changes in blood pressure.

PRES may be caused by several factors like in this patient, importantly severe hypertension as manifested by unstable vital signs or may be secondary to sepsis or drug-induced during her ICU admission.

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