J Cerebrovasc Endovasc Neurosurg.  2015 Sep;17(3):239-245. 10.7461/jcen.2015.17.3.239.

Reversible Cerebral Vasoconstriction Syndrome and Posterior Reversible Encephalopathy Syndrome Presenting with Deep Intracerebral Hemorrhage in Young Women

• Lee WJ
• Yeon JY
• Jo KI
• Kim JS
• Hong SC

• KMID: 2069250
• DOI: http://doi.org/10.7461/jcen.2015.17.3.239

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a group of syndromes characterized by reversible segmental constriction of cerebral arteries. Posterior reversible encephalopathy syndrome (PRES) is another clinical-radiologic syndrome characterized by reversible, posterior-predominant brain edema. Although the exact causes of these reversible syndromes are poorly understood, these entities may share some common pathophysiologic elements leading to hemorrhagic strokes and rarely, deep intracerebral hemorrhage (ICH). Recent studies have suggested that endothelial dysfunction is a common pathophysiologic factor associated with these syndromes. We report on two young female patients who presented with deep ICH and were later diagnosed as RCVS and PRES. Both patients suffered from vasoconstriction and delayed ischemic stroke. Early detection of distinguishing clinical-radiologic features associated with these reversible syndromes and removing triggers would facilitate successful treatment with no complications.

Keyword

Cerebral vasospasm; Cerebral arteries; Cerebral hemorrhage; Posterior reversible encephalopathy syndrome (PRES)

MeSH Terms

Brain Edema
Cerebral Arteries
Cerebral Hemorrhage*
Constriction
Female
Humans
Posterior Leukoencephalopathy Syndrome*
Stroke
Vasoconstriction*
Vasospasm, Intracranial

Figure

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  Fig. 1 (A) Brain CT shows the isolated deep ICH in the right basal ganglia. (B) Brain CTA shows multifocal narrowing of distal MCA branches (arrows). CT = computed tomography; ICH = intracerebral hemorrhage; CTA = CT angiography; MCA = middle cerebral arteries.

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  Fig. 2 MRI shows multifocal border zone infarctions. MRI = magnetic resonance imaging.

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  Fig. 3 (A) MRA shows the progression of multifocal vasoconstriction toward proximal cerebral arteries. (B) There is no wall enhancement of affected vessels on high resolution MR wall imaging. MRA = MR angiography; MR = magnetic resonance.

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  Fig. 4 Digital subtraction angiography demonstrates improving but residual vasoconstriction (arrows).

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  Fig. 5 Complete resolution of vasoconstriction is shown on 6-month follow-up MRA. MRA = MR angiography.

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  Fig. 6 (A) Brain CT shows deep ICH and associated IVH. Note the symmetrically scattered low density lesions in the bilateral basal ganglia and parietal lobes. (B) There is no vascular abnormality. CT = computed tomography; ICH = intracerebral hemorrhage; IVH = intraventricular hemorrhage.

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  Fig. 7 (A, B) T2 Fluid Attenuated Inversion Recovery (FLAIR) images (A) and apparent diffusion coefficient images (B) suggest vasogenic edema. (C) MRA is normal. MRA = MR angiography.

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  Fig. 8 (A) Follow-up MRI shows improved vasogenic edema. (B, C) Newly appeared multifocal stenoses are seen (B) with a new infarction (C). MRI = magnetic resonance imaging.

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  Fig. 9 Complete resolution of vasogenic edema and multifocal stenoses is shown on 3-month follow-up MRI (A) and MRA (B). MRI = magnetic resonance imaging; MRA = MR angiography.

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