The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)

Cephalalgia ◽  
2019 ◽  
Vol 39 (10) ◽  
pp. 1267-1276 ◽  
Author(s):  
Filomena Caria ◽  
Marialuisa Zedde ◽  
Massimo Gamba ◽  
Anna Bersano ◽  
Maurizia Rasura ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Neurological Complications ◽  
Young Age ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Cerebral Vasoconstriction ◽  
Disease Occurrence ◽  
Causative Factors

Introduction To describe clinical, neuroimaging, and laboratory features of a large cohort of Italian patients with reversible cerebral vasoconstriction syndrome. Methods In the setting of the multicenter Italian Project on Stroke at Young Age (IPSYS), we retrospectively enrolled patients with a diagnosis of definite reversible cerebral vasoconstriction syndrome according to the International Classification of Headache Disorders (ICHD)-3 beta criteria (6.7.3 Headache attributed to reversible cerebral vasoconstriction syndrome, imaging-proven). Clinical manifestations, neuroimaging, treatment, and clinical outcomes were evaluated in all patients. Characteristics of reversible cerebral vasoconstriction syndrome without typical causes (“idiopathic reversible cerebral vasoconstriction syndrome”) were compared with those of reversible cerebral vasoconstriction syndrome related to putative causative factors (“secondary reversible cerebral vasoconstriction syndrome”). Results A total of 102 patients (mean age, 47.2 ± 13.9 years; females, 85 [83.3%]) qualified for the analysis. Thunderclap headache at presentation was reported in 69 (67.6%) patients, and it typically recurred in 42 (60.9%). Compared to reversible cerebral vasoconstriction syndrome cases related to putative etiologic conditions (n = 21 [20.6%]), patients with idiopathic reversible cerebral vasoconstriction syndrome (n = 81 [79.4%]) were significantly older (49.2 ± 13.9 vs. 39.5 ± 11.4 years), had more frequently typical thunderclap headache (77.8% vs. 28.6%) and less frequently neurological complications (epileptic seizures, 11.1% vs. 38.1%; cerebral infarction, 6.1% vs. 33.3%), as well as concomitant reversible brain edema (25.9% vs. 47.6%). Conclusions Clinical manifestations and putative etiologies of reversible cerebral vasoconstriction syndrome in our series are slightly different from those observed in previous cohorts. This variability might be partly related to the coexistence of precipitating conditions with a putative etiologic role on disease occurrence.

Characteristics and demographics of reversible cerebral vasoconstriction syndrome: A large prospective series of Korean patients

Cephalalgia ◽  
2017 ◽  
Vol 38 (4) ◽  
pp. 765-775 ◽  
Author(s):  
Hyun Ah Choi ◽  
Mi Ji Lee ◽  
Hanna Choi ◽  
Chin-Sang Chung
Keyword(s):  
Medical Center ◽  
Clinical Manifestations ◽  
Neurological Complications ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Middle Aged ◽  
Cerebral Vasoconstriction ◽  
Female Predominance ◽  
Predominantly Female ◽  
Preceding Event

Objectives To report demographics and characteristics of reversible cerebral vasoconstriction syndrome (RCVS) in the Korean cohort. Methods We prospectively recruited patients with definite (imaging-proven) RCVS and probable (imaging-negative) RCVS who visited Samsung Medical Center between June 2012 and September 2016. Clinical manifestations, neuroimaging, treatment, and clinical outcomes were evaluated in all patients. Characteristics of RCVS without typical causes (“idiopathic RCVS”) were compared with those of RCVS with identifiable causes (“secondary RCVS”). International Classification of Headache Disorders (ICHD)-3 beta criteria for 6.7.3 RCVS and 6.7.3.1 probable RCVS were tested. Results A total of 138 patients (104 definite and 34 probable RCVS) were included in this study. Patients with definite RCVS were predominantly female (85.6%) and middle-aged (mean, 50.7 [range, 23–82] years). Probable RCVS was associated with less female predominance (70.6%, p = 0.049), more typical manifestations ( p < 0.001), and none of neurological complications. One-hundred and one (97.1%) patients with definite RCVS had headache, but the typical “recurrent and/or triggered” thunderclap headache was reported in only 83 (82.2%). In most patients with definite RCVS (84.6%), RCVS was idiopathic, while only 16 (15.4%) had secondary causes. Compared to those with secondary RCVS, patients with idiopathic RCVS were older (52.8 ± 11.42 vs. 39.1 ± 9.55 years, p < 0.001). Patients with secondary RCVS had more complications than those with idiopathic RCVS (40.5% vs. 12.5%, p = 0.018). Among idiopathic RCVS patients, 33 (37.5%) reported a preceding event or a change in lifestyle, environment, health, or medication within one month before onset. Conclusion In our cohort, RCVS was benign and idiopathic in most patients, and occurred frequently in middle-aged women. Manifestations of RCVS were more diverse than previously recognized, and forms without any headache existed. Different genetic, social, and environmental factors should be taken into account to unveil the spectrum and pathophysiology of RCVS.

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

Cephalalgia ◽  
2017 ◽  
Vol 38 (10) ◽  
pp. 1665-1671 ◽  
Author(s):  
Mi Ji Lee ◽  
Hyun Ah Choi ◽  
Hanna Choi ◽  
Chin-Sang Chung
Keyword(s):  
Medical Center ◽  
Final Diagnosis ◽  
Acute Stage ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Cerebral Vasoconstriction ◽  
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.

scholarly journals DIAGNOSTICS OF REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROME: CLINICAL MANIFESTATION AND IMAGING PATTERNS

2018 ◽  
pp. 50-55
Author(s):  
E. G. Klocheva ◽  
V. V. Goldobin
Keyword(s):  
Magnetic Resonance ◽  
Magnetic Resonance Angiography ◽  
Clinical Manifestation ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Treatment Modification ◽  
Cerebral Vasoconstriction

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.

scholarly journals Long-Term Outcomes After Reversible Cerebral Vasoconstriction Syndrome

Stroke ◽  
2020 ◽  
Vol 51 (2) ◽  
pp. 670-673 ◽  
Author(s):  
Rosalie Boitet ◽  
Solène de Gaalon ◽  
Claire Duflos ◽  
Grégory Marin ◽  
Jérôme Mawet ◽  
...  
Keyword(s):  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Long Term Outcomes ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Follow Up Study ◽  
Cerebral Vasoconstriction ◽  
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

2016 ◽  
Author(s):  
Ji Y. Chong ◽  
Michael P. Lerario
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Clinical Presentation ◽  
Clinical Symptoms ◽  
Aneurysmal Subarachnoid Hemorrhage ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Cerebral Vasoconstriction ◽  
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 ◽  
2017 ◽  
Vol 38 (5) ◽  
pp. 984-987 ◽  
Author(s):  
Björn Machner ◽  
Tobias Boppel ◽  
Thomas Münte
Keyword(s):  
Differential Diagnosis ◽  
Post Partum ◽  
Cerebral Arteries ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Gastrointestinal Infection ◽  
Cerebral Vasoconstriction ◽  
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.

scholarly journals Reversible cerebral vasoconstriction syndrome in a patient with COVID-19

2021 ◽  
Vol 26 (4) ◽  
pp. 829-834
Author(s):  
Arpan Dutta ◽  
Atanu Chandra ◽  
Subhadeep Gupta ◽  
Biman Kanti Ray ◽  
Deep Das ◽  
...  
Keyword(s):  
Cerebral Angiography ◽  
Rare Complication ◽  
Cerebral Arteries ◽  
Neurological Complications ◽  
Cerebrovascular Diseases ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Angiotensin Converting Enzyme 2 ◽  
Cerebral Vasoconstriction ◽  
Middle Cerebral Arteries ◽  
Novel Coronavirus

COVID-19 infection is well-known to produce different neurological complications, including cerebrovascular diseases. Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by transient segmental vasoconstriction of the cerebral vasculature, has been rarely reported in association with COVID-19 infection. The causative agent, the novel coronavirus (SARS-CoV-2), binds to the angiotensin-converting enzyme 2 (ACE-2) receptors for its entry into the host cell. This leads to downregulation of the ACE-2 and increased activity of the renin-angiotensin-aldosterone (RAAS) axis resulting in sympathetic overactivity and vasoconstriction. This might be the possible mechanism of RCVS in COVID-19. We hereby report a case of RCVS occurring in a SARS-CoV-2 infected patient. This was a 38-year-old male without any comorbidities or risk factors, who presented with headache and confusion. His SARS-CoV-2 RT-PCR was positive. MRI of the brain was normal but cerebral angiography revealed segmental vasoconstriction in bilateral middle cerebral arteries and the terminal part of the internal carotid arteries, which resolved almost completely after 2 weeks. He was treated with oral nimodipine 60 mg every 6 hourly. A database search revealed 2 previous cases of RCVS associated with COVID-19. In conclusion, RCVS is a rare complication of COVID-19. It is possibly under-recognized as only a few COVID-19 patients with headaches undergo cerebral angiography especially when parenchymal brain imaging is normal.

Sign in / Sign up

Export Citation Format

Share Document