Reversible Cerebral Vasoconstriction Syndrome Associated with Subarachnoid Hemorrhage

Abstract

Reversible cerebral vasoconstriction syndrome may be associated with subarachnoid hemorrhage. RCVS should be included in the differential diagnosis of non-aneurysmal subarachnoid hemorrhage.

Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

“…1,2,6,9,42,44 In patients with RCVS who have migraines, the thunderclap headache is typically described as differing in location, degree, and quality from their usual migraines. 13 A minority of patients with RCVS may present with a more mild or subacute headache, though the complete absence of headache is rare.…”

“…2,6,8,12,13,20,23,39,[57][58][59] In her meta-analysis of 3 large case series of patients with RCVS, Ducros 19 found that focal neurologic deficits were present in 8%-43% of patients, seizures in 1%-17%, cortical subarachnoid hemorrhage in 30%-34% (1 study had hemorrhage as an exclusion criterion and was not included), cerebral infarction in 6%-39%, and concomitant PRES in 9%-38% (Fig 2). This wide range in reported incidence of various sequelae of RCVS may reflect recruitment bias, with more ill patients being more likely to present for medical care; selection criteria; and the clinical context in which patients were encountered.…”

“…However, the diagnosis can be challenging because its signs and symptoms can overlap those of better known disorders of the central nervous system, including aneurysmal subarachnoid hemorrhage and primary angiitis of the CNS. 1,2,6,[12][13][14] Furthermore, a key feature of RCVS, segmental arterial vasoconstriction, may be absent early in the course of the disease. 1,2,4,5,14 Consequently, both the clinician and radiologist must maintain a high level of suspicion for this entity in patients presenting with characteristic features.…”

Hemorrhage in Posterior Reversible Encephalopathy Syndrome: Imaging and Clinical Features

“…Regions of both infarction and hemorrhage are also known to develop in the setting of cerebral vasoconstriction or vasospasm. 55,[57][58][59][60] In aneurysmal subarachnoid hemorrhage, early vasospasm can occur with infarction and both petechial blood and focal hematoma formation. 55 In the reversible cerebral vasoconstric-tion syndrome, watershed infarction and focal brain hematomas have been observed, potentially due to reperfusion.…”

“…60 In addition, similar to PRES, isolated sulcal subarachnoid hemorrhage has been seen in reversible cerebral vasoconstriction syndrome. 59 Hemorrhage has also been observed in the potentially related postinfectious or immune-triggered leukoencephalopathies. Although not typically seen on imaging studies in patients with multiple sclerosis, evidence of vein wall damage (intramural fibrinoid, collagenized thickening), recent or old hemorrhage (hemosiderin), and thrombosed vessels has been noted at histopathology in addition to the typical observations of perivenous immune cell response and demyelination.…”