Reversible Cerebral Vasoconstriction Syndrome Presenting Research Articles

Reversible Cerebral Vasoconstriction Syndrome Presenting as Micturition Headaches in a Pediatric Patient (P3-5.029)

Reversible Cerebral Vasoconstriction Syndrome Presenting as Micturition Headaches in a Pediatric Patient (P3-5.029)

Cyclophosphamide-Associated Reversible Cerebral Vasoconstriction Syndrome Presenting as Concurrent Cerebral Infarction and Subarachnoid Hemorrhage

Reversible cerebral vasoconstriction syndrome (RCVS) has several trigger factors, including physical exertion, pregnancy, and the intake of vasoconstrictive agents. These triggers activate the sympathetic nervous system and induce vasoconstriction, thereby leading to an ischemic or hemorrhagic stroke. In this study, we describe case of RCVS in a 73-year-old woman who complained of sudden bilateral leg weakness after taking cyclophosphamide for anti-neutrophil cytoplasmic antibody associated vasculitis. She was diagnosed with concurrent cerebral hemorrhage and cerebral infarction with multiple intracranial vasoconstrictions on imaging analyses.

The Spectrum of Nontraumatic Convexity Subarachnoid Hemorrhage

Background: Nontraumatic convexity subarachnoid hemorrhage (cSAH) is a nonaneurysmal variant that is associated with diverse etiologies. Methods: With IRB approval, we retrospectively reviewed consecutive nontraumatic cSAH from July 1, 2006 to July 1, 2016. Data were abstracted on demographics, medical history, neuroimaging, etiology, and clinical presentation. Results: We identified 94 cases of cSAH. The cases were classified according to the following etiologies: reversible cerebral vasoconstriction syndrome (RCVS) 17 (18%), cerebral amyloid angiopathy (CAA) 15 (16%), posterior reversible encephalopathy syndrome 16 (17%), cerebral venous thrombosis 10 (11%), large artery occlusion 7 (7%), endocarditis 6 (6%), and cryptogenic 25 (27%). Early rebleeding occurred in 9 (10%) patients. Time from initial imaging to CT rebleeding was 40 hours (range, 5-74). CAA was associated with the highest mean age at 75.8 and RCVS the lowest at 47.6 years (P< .0001). Among patients with RCVS, initial vascular imaging was negative in 6 (35%), and repeat imaging documented vasoconstriction at a mean delay of 5 days (range, 3-16). Conclusion: There were significant differences among the subgroups in cSAH, with CAA presenting as older men with transient neurological deficits, and RCVS presenting as younger women with thunderclap headache. Rebleeding was seen in 10% of cSAH patients. One-third of RCVS patients with cSAH required repeat vascular imaging to diagnose vasoconstriction.

Angiographic Characteristics of Hemorrhagic and Ischemic Phases of Reversible Cerebral Vasoconstriction Syndrome.

This study aimed to assess the evolution of imaging patterns over time in patients with neurological complications caused by reversible cerebral vasoconstriction syndrome. Atotal of 24 consecutive patients with reversible cerebral vasoconstriction syndrome presenting between 2009 and 2016 were included, whose disease course was complicated by intracranial hemorrhage and/or ischemic events. In total 55 angiographic studies were carried out. The nature of the intracranial complication and location of vasoconstriction on the angiograms in relation to the time interval since symptom-onset were assessed. Complications included subarachnoid hemorrhage (n = 19, 79%), intracerebral hemorrhage (n = 7, 29%), ischemic stroke (n = 6, 25%), and transient ischemic attack (n = 4, 17%). Hemorrhagic complications mainly occurred within 7days after symptom onset (18/19patients, 95%), whereas ischemic events only occurred after the first week (10/10patients, 100%, p < 0.00001). Distal vasospasm was predominantly observed within 7days (26/28angiograms, 93%) and proximal vasospasm ≥7days (23/27angiograms, 85%, p < 0.00001). In reversible cerebral vasoconstriction syndrome causing neurological complications, an early hemorrhagic phase with distal vasospasm and adelayed ischemic phase with proximal vasospasm can be discriminated.

Reversible cerebral vasoconstriction syndrome presenting as an isolated primary intraventricular hemorrhage

BackgroundPrimary intraventricular hemorrhage is an uncommon cause of stroke and is often associated with longstanding, uncontrolled hypertension. Reversible cerebral vasoconstriction is also an uncommon condition characterized by reversible constriction of intracerebral vessels, which can lead to ischemic or hemorrhagic strokes.Case presentationWe describe a case of isolated primary intraventricular hemorrhage secondary to reversible cerebral vasoconstriction syndrome triggered by pseudoephedrine.ConclusionsReversible cerebral vasoconstriction syndrome is a rare cause of primary intraventricular hemorrhage and should be considered in the differential in angiography-negative IVH when there is a history of vasoactive substance use.

Reversible Cerebral Vasoconstriction Syndrome Presenting as Third Cranial Nerve Palsy (P6.009)

Reversible Cerebral Vasoconstriction Syndrome Presenting as Third Cranial Nerve Palsy (P6.009)

Reversible Cerebral Vasoconstriction Syndrome Presenting with Bilateral Occipital Hemorrhage after the Subcutaneous Injection of Synthetic Gonadotropin Releasing Hormone

Reversible cerebral vasoconstriction syndrome (RCVS) is a heterogeneous group of cerebrovascular disease. The pathophysiology of RCVS is unknown, but a disturbance in cerebral vascular tone is one of hypothesis. Long-term use of Gonadotropin-releasing hormone (GnRH) agonists can induce a pseudomenopausal state in which estrogen production are suppressed. It might lead to reduced arterial relaxation by estrogen withdrawal. We report a case of RCVS after the injection of synthetic analogue of GnRH. Key Words: Reversible cerebral vasoconstriction syndrome, Intracerebral hemorrhage, Synthetic analogue of gonadotropin releasing hormone

Reversible Cerebral Vasoconstriction Syndrome presenting as Convexity Subarachnoid Hemorrhage (P2.286)

Reversible Cerebral Vasoconstriction Syndrome presenting as Convexity Subarachnoid Hemorrhage (P2.286)

Reversible Cerebral Vasoconstriction Syndrome Presenting as Coma (P4.354)

Reversible Cerebral Vasoconstriction Syndrome Presenting as Coma (P4.354)

Abstract TMP42: Distinguishing Clinical and Radiologic Features of Non-Traumatic Convexal Subarachnoid Hemorrhage

Introduction: Small case series have suggested that cerebral amyloid angiopathy (CAA) and reversible cerebral vasoconstriction syndrome (RCVS) are the most common causes of non-traumatic convexal SAH (cSAH). Our objective was to describe the spectrum of clinical and imaging features in a large series of non-traumatic cSAH patients. Methods: A retrospective observational study of consecutive patients with non-traumatic cSAH was performed. The Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated at Mayo Clinic in Rochester with potential non-traumatic cSAH between January 1, 1996 and October 1, 2014. Clinical and radiologic data were abstracted from the comprehensive medical record. Results: We identified 88 patients [median age, 64 years (range: 25-85)] with non-traumatic cSAH. The most common causes were reversible cerebral vasoconstriction syndrome (26, 29.5%), cerebral amyloid angiopathy (23, 26.1%), indeterminate (14, 15.9%), and endocarditis (9, 10.2%). Other causes include posterior reversible encephalopathy syndrome, cerebral vein thrombosis, vasculitis, carotid occlusive disease, thrombosed developmental venous anomaly, and hyperperfusion syndrome after carotid revascularization. CAA patients commonly presented at an older age than RCVS patients (75 years vs 51 years, p&lt;0.0001). The most common clinical presentation for CAA was sensorimotor dysfunction while thunderclap headache was the most common presentation of RCVS. Thirteen patients (14.7%) had recurrent cSAH and twelve patients (13.6%) had a subsequent intracerebral hemorrhage over a median follow up of 16 months. The risk was high among CAA patients, with subsequent lobar ICH occurring in 43.5% of cases, superficial siderosis in 95%, and recurrent cSAH in 39.1%, compared to 3.8%, 28.5% and 7.7% respectively in RCVS. Conclusions: Our study demonstrates the clinical diversity of cSAH. Older age, sensorimotor dysfunctions suggest CAA as the underlying cause. Younger age and thunderclap headache predict RCVS. Various other causes also need to be considered in the differential diagnosis. CAA patients have high rates of subsequent lobar ICH, development of superficial siderosis, and recurrent cSAH.

An unusual case of reversible cerebral vasoconstriction syndrome presenting during antepartum period

An unusual case of reversible cerebral vasoconstriction syndrome presenting during antepartum period

Reversible Cerebral Vasoconstriction Syndrome: Treatment with Multiple Sessions of Intra-Arterial Nimodipine and Angioplasty

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe headache with or without additional neurological symptoms and reversible cerebral vasoconstriction. Unruptured aneurysms have been reported in some cases with RCVS. We describe a severe case of a 50-year-old woman with RCVS presenting as cortical subarachnoid hemorrhage. Cerebral angiogram demonstrated the typical angiographic findings of RCVS and two very small unruptured aneurysms of the left internal carotid artery. The patient was treated with calcium channel blockers and the two aneurysms were successfully treated endovascularly. On day 16 the patient developed new focal neurological symptoms (severe paraparesis) and was successfully treated with intraarterial nimodipine and angioplasty in multiple sessions.

Reversible Cerebral Vasoconstriction Syndrome Presenting as Cortical Subarachnoid Hemorrhage

Reversible Cerebral Vasoconstriction Syndrome Presenting as Cortical Subarachnoid Hemorrhage

Isolated thunderclap headache during sex: Orgasmic headache or reversible cerebral vasoconstriction syndrome?

Orgasmic headache (OH) is an “explosive” headache that occurs at orgasm. Historically, it was considered benign with no treatment needed. Reversible cerebral vasoconstriction syndrome (RCVS) refers to a group of disorders characterized by recurrent thunderclap headache (TCH) and multifocal vasoconstriction. Patients who have RCVS often recover completely, but some may have persistent neurological deficits. We report a 34-year-old woman who presented with isolated and recurrent TCH at orgasm, which fulfilled the diagnosis of OH. However, she was post-partum and had recent exposure to ecstasy, making her symptoms highly suggestive of RCVS. Brain magnetic resonance angiography showed segmental vasoconstriction. We concluded that she could be considered to have either OH or RCVS. This patient suggests the theory that OH could be a presentation of RCVS. Given that RCVS is potentially treatable, early recognition by clinicians is vital in order to prevent devastating complications.