Fulminant Reversible Cerebral Vasoconstriction Syndrome: The Other Thunderclap Headache

Treatment Modification ◽

Data of 130 patients with thunderclap headache are presented. The data include clinical manifestation analysis and neuroimaging results (magnetic resonance angiography). Magnetic resonance angiography was performed 15 days after acute clinical manifestation and permitted to verify cerebral vasoconstriction, that led to treatment modification with clinical and neuroimaging signs of vasoconstriction reverse.

Long-Term Outcomes After Reversible Cerebral Vasoconstriction Syndrome

Stroke ◽

10.1161/strokeaha.119.027703 ◽

2020 ◽

Vol 51 (2) ◽

pp. 670-673 ◽

Cited By ~ 4

Author(s):

Rosalie Boitet ◽

Solène de Gaalon ◽

Claire Duflos ◽

Grégory Marin ◽

Jérôme Mawet ◽

...

Keyword(s):

Thunderclap Headache ◽

Long Term Outcomes ◽

Term Outcome ◽

Long Term Outcome ◽

Follow Up Study ◽

History Of

Background and Purpose— We aimed to further investigate the long-term outcomes after reversible cerebral vasoconstriction syndrome (RCVS). Methods— A longitudinal follow-up study was conducted in 173 RCVS patients. Results— Of the 172 patients who completed a mean follow-up of 9.2±3.3 years, 10 had a recurrent RCVS that was benign in all. Independent predictors of relapse were having a history of migraine and having exercise as a trigger for thunderclap headache during initial RCVS. After new delivery, the rate of postpartum RCVS was 9%. Conclusions— Overall, long-term outcome after RCVS is excellent.

Thunderclap Headache

10.1093/med/9780190495541.003.0024 ◽

2016 ◽

Author(s):

Ji Y. Chong ◽

Michael P. Lerario

Keyword(s):

Subarachnoid Hemorrhage ◽

Clinical Presentation ◽

Clinical Symptoms ◽

Aneurysmal Subarachnoid Hemorrhage ◽

Thunderclap Headache ◽

Vasoactive Medication ◽

Angiographic Findings

Reversible cerebral vasoconstriction syndrome can cause thunderclap headache, subarachnoid hemorrhage, and stroke. The clinical presentation can be similar to aneurysmal subarachnoid hemorrhage and therefore requires rapid angiography. Angiographic findings of vasculopathy and resolution of clinical symptoms and imaging abnormalities support this diagnosis. This syndrome is most often incited following pregnancy or the ingestion of a vasoactive medication.

Isoflavones and gastrointestinal infection: Two potential triggers for reversible cerebral vasoconstriction syndrome

Cephalalgia ◽

10.1177/0333102417714245 ◽

2017 ◽

Vol 38 (5) ◽

pp. 984-987 ◽

Cited By ~ 1

Author(s):

Björn Machner ◽

Tobias Boppel ◽

Thomas Münte

Keyword(s):

Differential Diagnosis ◽

Post Partum ◽

Cerebral Arteries ◽

Thunderclap Headache ◽

Gastrointestinal Infection ◽

Vasoactive Substances ◽

Important Differential Diagnosis ◽

Estrogenic Potential

Background Reversible cerebral vasoconstriction syndrome (RCVS) is an important differential diagnosis of singular or recurrent thunderclap headache. Prognosis is generally good, however complications of the transient segmental vasospasms of cerebral arteries such as stroke, subarachnoidal hemorrhage and brain edema may worsen the clinical outcome. Although the exact pathomechanism is still unclear, various vasoactive substances and conditions (e.g. post partum) have been identified as triggering RCVS. Cases We report on the clinical course and management of two cases of typical RCVS that were associated with two different precipitants previously not described: A gastrointestinal infection and isoflavones, which are phytoestrogens used for menopausal vasomotor symptoms. Discussion In the case of gastrointestinal infection, either systemic inflammatory processes might lead to disturbances of vascular tone, or the repetitive vomiting that resembles Valsalva manoeuvers known to trigger RCVS. In the case of isoflavone intake, it may be their estrogenic potential that induces dysregulation of cerebral arteries, a mechanism known from other states of hormonal change such as post-partum angiopathy. However, the association of both precipitating factors with RCVS in our two cases is not a proof for a causal relationship, and there may have been additional potential triggers for RCVS. Conclusion In patients with (gastrointestinal) infection and concomitant thunderclap headache, RCVS should be considered as an important differential diagnosis due to its major complications. Since RCVS may be triggered by various vasoactive substances, taking the medical history should always include over-the-counter drugs and dietary supplements (such as the isoflavones) beside the regular medication.

Reversible cerebral vasoconstriction syndrome manifesting as focal seizures without a thunderclap headache: A pediatric case report

Brain and Development ◽

10.1016/j.braindev.2016.04.006 ◽

2016 ◽

Vol 38 (9) ◽

pp. 880-883 ◽

Cited By ~ 2

Author(s):

Shuji Kuga ◽

Hironori Goto ◽

Kazuo Okanari ◽

Tomoki Maeda ◽

Kenji Ihara

Keyword(s):

Case Report ◽

Thunderclap Headache ◽

Pediatric Case ◽

Focal Seizures ◽

Quadruple Extracranial Arterial Dissection with Reversible Cerebral Vasoconstriction Syndrome (RCVS) in a Habitual Cocaine User Presenting with Thunderclap Headache (P03.216)

Neurology ◽

10.1212/wnl.78.1_meetingabstracts.p03.216 ◽

2012 ◽

Vol 78 (Meeting Abstracts 1) ◽

pp. P03.216-P03.216

Author(s):

J. Morren ◽

R. Govindarajan ◽

A. Soriano Caminero ◽

D. Jones ◽

A. Rodriguez ◽

...

Keyword(s):

Thunderclap Headache ◽

Arterial Dissection ◽

Cocaine User ◽

Serial testing of the ICHD-3 beta diagnostic criteria for probable reversible cerebral vasoconstriction syndrome: A prospective validation study

Cephalalgia ◽

10.1177/0333102417744361 ◽

2017 ◽

Vol 38 (10) ◽

pp. 1665-1671 ◽

Cited By ~ 2

Author(s):

Mi Ji Lee ◽

Hyun Ah Choi ◽

Hanna Choi ◽

Chin-Sang Chung

Keyword(s):

Medical Center ◽

Final Diagnosis ◽

Acute Stage ◽

Thunderclap Headache ◽

Diagnostic Work ◽

Work Up ◽

Beta Version

Objectives To serially test the International Classification of Headache Disorders (ICHD) 3rd edition beta version criteria for 6.7.3.1 probable reversible cerebral vasoconstriction syndrome (probable RCVS) in patients with thunderclap headache. Methods We prospectively screened consecutive patients with thunderclap headache who visited the Samsung Medical Center between October 2015 and March 2017. Patients were included in the analysis if they a) visited our hospital within 1 month after onset, b) completed a diagnostic work-up, and c) had no secondary causes other than RCVS. The ICHD-3 beta 6.7.3.1 criteria were evaluated serially during the first visit (visit 1), 2 weeks after the first visit (visit 2), and 1–3 months after onset (visit 3). Results A total of 99 patients completed this study. Based on the first clinical and radiological evaluation, 63 (63.6%) were diagnosed with angiogram-proven RCVS, whilst 36 (36.4%) showed normal neuroimaging. The ICHD-3 beta 6.7.3.1 criteria were fulfilled in 76.2% of patients with angiogram-proven RCVS. In patients with normal angiograms, the ICHD diagnosis of probable RCVS changed over time: 25.0%, 47.2%, and 38.9% at visits 1, 2, and 3, respectively. The sensitivity and specificity of the criteria at visit 1 were 72.4% and 95.5%, respectively, for the prediction of a final diagnosis of overall (angiogram-proven + probable) RCVS. Conclusions The ICHD-3 beta criteria for probable RCVS are applicable for patients with thunderclap headaches at the acute stage, with the exception of criterion C3. Criteria C1 and C2 are less reliable when applied at the first visit. Repeated evaluation is necessary to enhance diagnostic sensitivity.

Diagnostic Value of Thunderclap Headache and Convexal Subarachnoid Hemorrhage for Reversible Cerebral Vasoconstriction Syndrome: A Case Report

Cureus ◽

10.7759/cureus.20411 ◽

2021 ◽

Author(s):

Ricardo D Otiniano-Sifuentes ◽

Laura Zelada-Ríos ◽

Jorge Ramírez-Quiñones ◽

Carlos Abanto ◽

María Novoa ◽

...

Keyword(s):

Case Report ◽

Subarachnoid Hemorrhage ◽

Diagnostic Value ◽

Thunderclap Headache ◽

Convexal Subarachnoid Hemorrhage

Thunderclap headache and reversible cerebral vasoconstriction syndrome: Current theory and accumulated data

Neurology and Clinical Neuroscience ◽

10.1111/ncn3.12347 ◽

2019 ◽

Vol 8 (3) ◽

pp. 139-145

Author(s):

Shoji Kikui ◽

Junichi Miyahara ◽

Daisuke Danno ◽

Yoshihiro Kashiwaya ◽

Takao Takeshima

Keyword(s):

Current Theory ◽

Thunderclap Headache ◽

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

Stroke ◽

10.1161/str.47.suppl_1.tmp42 ◽

2016 ◽

Vol 47 (suppl_1) ◽

Author(s):

Jonathan Graff-Radford ◽

Jennifer Fugate ◽

James Klaas ◽

Kelly Flemming ◽

Robert Brown ◽

...

Keyword(s):

Cerebral Amyloid Angiopathy ◽

Mayo Clinic ◽

Older Age ◽

Thunderclap Headache ◽

Superficial Siderosis ◽

Amyloid Angiopathy ◽

Common Causes ◽

Cerebral Amyloid

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<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.