scholarly journals Coexisting vascular lesions in reversible cerebral vasoconstriction syndrome

Cephalalgia ◽  
2016 ◽  
Vol 37 (1) ◽  
pp. 29-35 ◽  
Author(s):  
M Akif Topcuoglu ◽  
Oguzhan Kursun ◽  
Aneesh B Singhal

Background The pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS) is not known. Published cases have documented coexisting cervical artery dissection and unruptured aneurysms, raising the possibility that ultrastructural vessel wall abnormalities underlie the development of vascular lesions as well as RCVS. Methods In this retrospective study we compared the frequency of neurovascular abnormalities in 158 consecutive RCVS patients, 44 patients with primary angiitis of the central nervous system (PACNS, positive controls), and 177 non-stroke patients with acute neurological symptoms (non-arteriopathy controls). Results Coexisting neurovascular abnormalities were significantly higher ( p < 0.001) in RCVS (23%) as compared to the PACNS (5%) or non-arteriopathy groups (8%). Cervical artery dissections were noted only in the RCVS group (8%, p < 0.001). The RCVS group had more unruptured aneurysms than PACNS (13% vs. 5%, p = 0.099) or non-arteriopathy controls (13% vs. 7%, p = 0.05). Seven RCVS patients also had other vascular malformations (venous anomaly, cavernous malformations, fibromuscular dysplasia). There was no significant association between coexisting vascular abnormalities and brain lesions or discharge clinical outcome in the RCVS group. Conclusion The high prevalence and heterogeneous anatomy of coexisting vascular lesions suggest that subtle ultrastructural arterial wall abnormalities may contribute to their development and also predispose to RCVS.

Neurology ◽  
2013 ◽  
Vol 81 (9) ◽  
pp. 821-824 ◽  
Author(s):  
J. Mawet ◽  
M. Boukobza ◽  
J. Franc ◽  
M. Sarov ◽  
M. Arnold ◽  
...  

Neurology ◽  
2018 ◽  
Vol 91 (16) ◽  
pp. e1468-e1478 ◽  
Author(s):  
Hubert de Boysson ◽  
Jean-Jacques Parienti ◽  
Jérôme Mawet ◽  
Caroline Arquizan ◽  
Grégoire Boulouis ◽  
...  

ObjectivesTo further improve the distinction between primary angiitis of the CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS).MethodsWe compared 2 large French cohorts of patients with PACNS (n = 110, retrospectively and prospectively enrolled) and RCVS (n = 173, prospectively enrolled).ResultsPatients with RCVS were predominantly female (p < 0.0001), with migraines (p < 0.0001), and were more often exposed to vasoactive substances (p < 0.0001) or postpartum (p = 0.002) than patients with PACNS. Headache, especially thunderclap headache, was more frequent in RCVS (both p < 0.0001). Thunderclap headache was absent in only 6% of patients with RCVS and was mainly recurrent (87%) and provoked (77%) mostly by sexual intercourse, exertion, or emotion. All other neurologic symptoms (motor deficit, seizure, cognitive disorder, or vigilance impairment, all p < 0.0001) were more frequent in PACNS. At admission, brain CT or MRI was abnormal in all patients with PACNS and in 31% of patients with RCVS (p < 0.0001). Acute ischemic stroke was more frequent in PACNS than in RCVS (p < 0.0001). Although intracerebral hemorrhage was more frequent in PACNS (p = 0.006), subarachnoid hemorrhage and vasogenic edema predominated in RCVS (p = 0.04 and p = 0.01, respectively). Multiple small deep infarcts, extensive deep white matter lesions, tumor-like lesions, or multiple gadolinium-enhanced lesions were observed only in PACNS, whereas cervical artery dissection was found only in RCVS.ConclusionsOur study confirms that careful analysis of clinical context, headache features, and patterns of brain lesions can distinguish PACNS and RCVS within the first few days of admission in most cases. However, diagnosis remains challenging in a few cases.


2020 ◽  
Vol 40 (03) ◽  
pp. 294-302
Author(s):  
William Qubty ◽  
Samantha Lee Irwin ◽  
Christine K. Fox

AbstractReversible cerebral vasoconstriction syndrome (RCVS) is a clinical–radiologic diagnosis that affects children and adolescents, but it is much more frequently reported in adults. Clinically, patients present with severe and commonly recurrent thunderclap headaches. Typical precipitating triggers include vasoactive substances, serotonergic agents, and the postpartum period. There may be associated neurologic complications at presentation or in the weeks following, such as convexity subarachnoid hemorrhage, stroke, cerebral edema, cervical artery dissection (CeAD), and seizures. Angiographically, the cerebral arteries demonstrate segmental vasoconstriction and dilation, although imaging early in the clinical course may be normal. Work-up is performed to exclude intracranial disorders such as vasculitis, subarachnoid hemorrhage due to ruptured aneurysm, meningitis, and intracranial venous sinus thrombosis. Within 1 month of initial symptom onset, clinical symptoms such as severe headache have ceased, and within 3 months, the cerebral vasoconstriction is much improved or resolved. Management involves avoidance of precipitating triggers and potentially short-term pharmacotherapy with calcium channel blockers for patients with associated neurologic complications. Steroids are not recommended and may worsen the clinical outcome. Prognosis is excellent in the large majority of patients, and only 5% of patients experience a recurrence of RCVS.


2020 ◽  
Vol 88 (3) ◽  
pp. 596-602
Author(s):  
Setareh Salehi Omran ◽  
Neal S. Parikh ◽  
Sharon Poisson ◽  
Jennifer Armstrong ◽  
Alexander E. Merkler ◽  
...  

Author(s):  
Guilherme Finger ◽  
Tobias Ludwig do Nascimento ◽  
Julia Carolina Lusa Tessaro ◽  
Eduarda Tanus Stefani ◽  
Maria Eduarda Conte Gripa ◽  
...  

AbstractEven though traumatic dissection of cervical arterial vessels is the major cause of stroke among adults, it is still an underdiagnosed disease in neurosurgical emergencies, since most patients do not have or present subtle clinical signs in the acute phase. The authors report two interesting cases of cervical artery dissection with different traumatic mechanisms and present a broad literature review about this subject.


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