J Korean Neurosurg Soc.  2014 Nov;56(5):419-422. 10.3340/jkns.2014.56.5.419.

Progressive Manifestations of Reversible Cerebral Vasoconstriction Syndrome Presenting with Subarachnoid Hemorrhage, Intracerebral Hemorrhage, and Cerebral Infarction

• Choi KS
• Yi HJ

• KMID: 2018101
• DOI: http://doi.org/10.3340/jkns.2014.56.5.419

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset headache with focal neurologic deficit and prolonged but reversible multifocal narrowing of the distal cerebral arteries. Stroke, either hemorrhagic or ischemic, is a relatively frequent presentation in RCVS, but progressive manifestations of subarachnoid hemorrhage, intracerebral hemorrhage, cerebral infarction in a patient is seldom described. We report a rare case of a 56-year-old woman with reversible cerebral vasoconstriction syndrome consecutively presenting as cortical subarachnoid hemorrhage, intracerebral hemorrhage, and cerebral infarction. When she complained of severe headache with subtle cortical subarachnoid hemorrhage, her angiography was non-specific. But, computed tomographic angiography showed typical angiographic features of this syndrome after four days. Day 12, she suffered mental deterioration and hemiplegia due to contralateral intracerebral hematoma, and she was surgically treated. For recurrent attacks of headache, medical management with calcium channel blockers has been instituted. Normalized angiographic features were documented after 8 weeks. Reversible cerebral vasoconstriction syndrome should be considered as differential diagnosis of non-aneurysmal subarachnoid hemorrhage, and repeated angiography is recommended for the diagnosis of this under-recognized syndrome.

Keyword

Reversible cerebral vasoconstriction syndrome; Angiography; Subarachnoid hemorrhage; Intracerebral hemorrhage; Cerebral infarction

MeSH Terms

Angiography
Calcium Channel Blockers
Cerebral Arteries
Cerebral Hemorrhage*
Cerebral Infarction*
Diagnosis
Diagnosis, Differential
Female
Headache
Hematoma
Hemiplegia
Humans
Middle Aged
Neurologic Manifestations
Stroke
Subarachnoid Hemorrhage*
Vasoconstriction*
Calcium Channel Blockers

Figure

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  Fig. 1 A : Initial head CT scan shows subtle cortical subarachnoid hemorrhage overlying the right high frontal convexity. B : Digital subtraction angiography shows no aneurysms or arteriovenous malformation or other arterial caliber change.

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  Fig. 2 Repeated CT angiographic images after 4 days on admission demonstrate prominent multifocal segmental vasoconstrictions involving superior division of both middle cerebral arteries (M2 tracts) and right posterior cerebral artery (P2 tract).

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  Fig. 3 A : Non-enhanced head CT at day 12 after admission shows intracerebral hemorrhage with edema on left cerebral hemisphere, associated with neurologic deterioration of the patient. B : On postoperative 12th day, CT scan reveals decreased extent of edema and hemorrhage.

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  Fig. 4 The MR diffusion-weighted image on day 37 shows a hyperintense signal in the pons, suggestive of acute or subacute infarction.

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  Fig. 5 Images of CT angiography (A) at 6 weeks and digital subtraction angiography (B and C) at 8weeks after initial hemorrhage demonstrate near complete resolution of segmental vasoconstriction previously seen in the both middle cerebral arteries and right posterior cerebral artery, respectively.

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