Reversible Cerebral Vasoconstriction Syndrome: A Novel Mechanism for Neurological Complications in Schimke Immuno-osseous Dysplasia

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.

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Reversible cerebral vasoconstriction syndrome in a patient with COVID-19

Neurology Asia ◽

10.54029/2021auv ◽

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.

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Reversible Cerebral Vasoconstriction Syndrome: An Important Cause of Acute Severe Headache

Emergency Medicine International ◽

10.1155/2012/303152 ◽

2012 ◽

Vol 2012 ◽

pp. 1-8 ◽

Cited By ~ 6

Author(s):

Li Huey Tan ◽

Oliver Flower

Keyword(s):

Central Nervous System ◽

Neurological Complications ◽

Severe Headache ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Diagnostic Features ◽

Therapeutic Implications ◽

Cerebral Vasoconstriction ◽

Primary Angiitis ◽

The Central Nervous System ◽

Acute Headache

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.

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

Cephalalgia ◽

10.1177/0333102419849013 ◽

2019 ◽

Vol 39 (10) ◽

pp. 1267-1276 ◽

Cited By ~ 2

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.

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