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

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.

Cephalalgia ◽  
2019 ◽  
Vol 39 (10) ◽  
pp. 1267-1276 ◽  
Author(s):  
Filomena Caria ◽  
Marialuisa Zedde ◽  
Massimo Gamba ◽  
Anna Bersano ◽  
Maurizia Rasura ◽  
...  

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.


Cephalalgia ◽  
2017 ◽  
Vol 38 (10) ◽  
pp. 1665-1671 ◽  
Author(s):  
Mi Ji Lee ◽  
Hyun Ah Choi ◽  
Hanna Choi ◽  
Chin-Sang Chung

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.


2021 ◽  
Vol 26 (4) ◽  
pp. 829-834
Author(s):  
Arpan Dutta ◽  
Atanu Chandra ◽  
Subhadeep Gupta ◽  
Biman Kanti Ray ◽  
Deep Das ◽  
...  

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.


2020 ◽  
pp. 22-26
Author(s):  
M. Yu. Afanasyeva ◽  
V. V. Goldobin ◽  
E. G. Klocheva

The data of patients with reversible cerebral vasoconstriction syndrome (RCVS) who were examined and treated in 2013–2020 are presented. 136 patients were examined (37.3 ± 11.4 years) – 107 (78.7 %) women, 29 (21.3 %) men. Detailed neurological examination, magnetic resonance imaging of the brain, magnetic resonance angiography and magnetic resonance venography were performed. Results. Primary RCVS was detected in 29 (21.3%), secondary RCVS – in 69 patients (50.7%), and a combination of primary and secondary RCVS factors – in 38 (28.0%) patients. In 95 (69.8%) patients (80 women, 15 men; p < 0.05) signs of cerebral artery angiodysplasia were identified. Hypoplasia of venous sinuses (transverse and/or sigmoid) was found in 34 patients (29 women and 5 men). Conclusions. RCVS is developed in women more often than men, but there was no significant difference in risk factors between men and women. Predisposing factors of both primary and secondary RCVS can be revealed simultaneously. Women were more likely to have variants of brachiocephalic artery development. Clarification of angiodysplasia variant influence to the development and severity of RCVS requires further study.


2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Li Huey Tan ◽  
Oliver Flower

Reversible cerebral vasoconstriction syndrome (RCVS) is an increasingly recognized and important cause of acute headache. The majority of these patients develop potentially serious neurological complications. Rigorous investigation is required to exclude other significant differential diagnoses. Differentiating RCVS from subarachnoid haemorrhage (SAH) and primary angiitis of the central nervous system (PACNS) may be difficult but has important therapeutic implications. This paper describes what is currently known about the epidemiology, pathophysiology, clinical, and diagnostic features of the syndrome, an approach to investigation, a summary of treatments, and what is known of prognosis.


Vaccines ◽  
2021 ◽  
Vol 9 (5) ◽  
pp. 497
Author(s):  
Helena C. Maltezou ◽  
Androula Pavli ◽  
Athanasios Tsakris

Post-COVID syndrome is increasingly recognized as a new clinical entity in the context of SARS-CoV-2 infection. Symptoms persisting for more than three weeks after the diagnosis of COVID-19 characterize the post-COVID syndrome. Its incidence ranges from 10% to 35%, however, rates as high as 85% have been reported among patients with a history of hospitalization. Currently, there is no consensus on the classification of post-COVID syndrome. We reviewed the published information on post-COVID syndrome, putting emphasis on its pathogenesis. The pathogenesis of post-COVID syndrome is multi-factorial and more than one mechanism may be implicated in several clinical manifestations. Prolonged inflammation has a key role in its pathogenesis and may account for some neurological complications, cognitive dysfunction, and several other symptoms. A multisystem inflammatory syndrome in adults (MIS-A) of all ages has been also described recently, similarly to multisystem inflammatory syndrome in children (MIS-C). The post-infectious inflammatory pathogenetic mechanism of MIS-A is supported by the fact that its diagnosis is established through serology in up to one third of cases. Other pathogenetic mechanisms that are implicated in post-COVID syndrome include immune-mediated vascular dysfunction, thromboembolism, and nervous system dysfunction. Although the current data are indicating that the overwhelming majority of patients with post-COVID syndrome have a good prognosis, registries to actively follow them are needed in order to define the full clinical spectrum and its long-term outcome. A consensus-based classification of post-COVID syndrome is essential to guide clinical, diagnostic, and therapeutic management. Further research is also imperative to elucidate the pathogenesis of post-COVID syndrome.


Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Karlo Toljan ◽  
Monica Scarsella ◽  
Meghan Purohit ◽  
Dolora R Wisco ◽  
Ken Uchino

Introduction: Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) might represent a pathophysiological spectrum. They are commonly diagnosed separately, based on prominent clinical features. We aimed to compare clinical and radiographic findings of PRES and RCVS. Methods: We performed a retrospective study of adult patients admitted to a tertiary medical center from February 2008 until February 2018 and were diagnosed with PRES or RCVS. Patient demographics, risk factors, clinical features, imaging, and outcomes were compared. Appropriate statistical tests were used to compare the variables and significant findings are reported. Results: There were 281 PRES and 98 RCVS cases meeting diagnostic criteria. Seizures, encephalopathy, and hypertension were more common with PRES, whereas headache was more common with RCVS (p <0.001). Hypertension and transplant recipient or immunocompromised status were associated with PRES (p <0.001), as did diabetes mellitus (p <0.05). Marijuana use, smoking, and obesity were associated with RCVS (p <0.05). ). Intraparenchymal or subarachnoid hemorrhage was found in 51 PRES cases and 34 RCVS cases. Arterial stenosis was present in 19 % of PRES cases. Brain FLAIR MRI hyperintensity that was reversible on follow-up was present in 26% of RCVS cases. Conclusion: PRES and RCVS share common clinical characteristics and might represent a pathophysiological spectrum, though distinct clinical features were noted in our retrospective analysis.


2019 ◽  
Vol 1 (7) ◽  
pp. 19-23
Author(s):  
S. I. Surkichin ◽  
N. V. Gryazeva ◽  
L. S. Kholupova ◽  
N. V. Bochkova

The article provides an overview of the use of photodynamic therapy for photodamage of the skin. The causes, pathogenesis and clinical manifestations of skin photodamage are considered. The definition, principle of action of photodynamic therapy, including the sources of light used, the classification of photosensitizers and their main characteristics are given. Analyzed studies that show the effectiveness and comparative evaluation in the selection of various light sources and photosensitizing agents for photodynamic therapy in patients with clinical manifestations of photodamage.


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