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

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.

Hemorrhagic Reversible Cerebral Vasoconstriction Syndrome: Clinical Presentation, Differential Diagnosis, Mechanism, and Treatment

“…RCVS has a clinical presentation of thunderclap headache, nausea, vomiting, photophobia, encephalopathy, seizure, focal neurologic deficit due to ischemia, cerebral edema, and cerebral infarction 3) . An initial DSA and CTA are the preferred imaging modalities, and such images show multifocal segmental vasoconstriction and reversibility of angiographic spasms in the three months following the onset of clinical symptoms 4) .…”

“…An initial DSA and CTA are the preferred imaging modalities, and such images show multifocal segmental vasoconstriction and reversibility of angiographic spasms in the three months following the onset of clinical symptoms 4) . Although the pathophysiology is still not yet clearly defined, multiple factors can cause SAH including pial vessels and reperfusion injury due to high flow in spastic vessels 3) . Sympathetic hyperreactivity by medication, endothelial dysfunction, and oxidative stress are prone to generating vasoconstriction, causing RCVS 4) .…”

Decompression Hemicraniectomy for Refractory Intracranial Hypertension in Reversible Cerebral Vasoconstriction Syndrome

“…Hemorrhagic RCVS occurs in one-third of RCVS patients and some with associated diffuse cerebral edema due to multifocal ICH, cSAH, and PRES changes. [6] The initial ICH burden predicts the outcome and thus preventing secondary brain injury from intracranial hypertension is of paramount importance to minimize disability. Singhal et al ., in their pooled analysis of 139 cases, found 9% of patients had unfavorable outcome (modified Rankin Scale 4–6) with 2% mortality due to severe vasoconstriction.…”

“…[5] Hemorrhagic complications, such as ICH and cSAH, occur early in the course secondary to severe vasospasm of smaller arteries, followed by progressive centripetal vasoconstriction of larger arteries leading to infarction. [56] Cerebral edema is an underrecognized complication in RCVS. It can be secondary to ischemic and hemorrhagic stroke, SAH, PRES, vasospasm with subclinical ischemia, hydrocephalus, or disruption of the blood–brain barrier.…”