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CASE REPORT
Year : 2022 | Volume
: 8
| Issue : 4 | Page : 222-224
Extracranial reversible cerebral vasoconstriction syndrome associated with vertebral artery dissection: A case report
Miyeon Yoon, Taewon Kim
Department of Neurology, Incheon St. Mary's Hospital, The Catholic University of Korea, Incheon, Korea
Correspondence Address:
Taewon Kim
Department of Neurology, Incheon St. Mary's Hospital, The Catholic University of Korea, #56 Dongsu-ro, Bupyeong-gu, Incheon 21431
Korea
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/bc.bc_58_22
In the present vignette, we describe a lateral medullary infarction developed immediately after strenuous straining owing to constipation in a 42-year-old female. There was a dissection in left vertebral artery V4 segment. Computed tomography (CT) angiography revealed beaded appearance of cervical V2 and V3 segments of bilateral vertebral arteries. A follow-up CT angiogram performed about 3 months later showed resolution of vasoconstriction and normalization of vertebral arteries. Reversible cerebral vasoconstriction syndrome (RCVS) is usually known as an intracranial pathologic condition. Extracranial RCVS is very rare. Therefore, the diagnosis of RCVS could be challenging when its location is extracranial, particularly when comingling vertebral artery dissection (VAD) is present because of their similar vascular luminal morphology. Physician should be vigilant about the possibility of a concomitant presence of RCVS and VAD, even in extracranial vessels.
Keywords: Dissection, reversible cerebral vasoconstriction syndrome, vasospasm
Reversible cerebral vasoconstriction syndrome (RCVS) is a distinct vasculopathy characterized by thunderclap headaches and transient reversible cerebral vasoconstriction. RCVS has been associated with various triggering factors of structural brain/vascular lesions including intracranial hemorrhage, posterior reversible encephalopathy syndrome, cervical artery dissection, and aneurysm.[1] In addition, nonstructural triggering factors such as pregnancy, migraine, vasoconstrictive drugs, orgasm, Valsalva maneuvers, or physical exertion have been reported to be implicated in the development of RCVS.[1],[2],[3]
RCVS was originally considered to be a disease of the intracranial vasculature. Its extracranial involvement is scarce.[1],[2],[3] Herein, we present a patient with RCVS involving bilateral extracranial vertebral arteries associated with intracranial vertebral artery dissection (VAD).
Case ReportA 42-year-old female presented to the emergency room with sudden-onset vertigo, diplopia, and acutely severe headache immediately after a strenuous straining owing to constipation. She had no other relevant medical history. Neurological examination revealed right-beating spontaneous nystagmus with a clockwise rotatory component, left facial hypesthesia with light touch sensation, limb dysmetria in the left arm, and left veering/falling tendency.
Diffusion-weighted magnetic resonance imaging (MRI) of the brain showed a left lateral medullary infarction [Figure 1]a. MRI T1 sequence revealed a hyperintense lesion within the left vertebral artery [Figure 1]b, in which susceptibility was correspondingly observed on susceptibility-weighted image MRI [Figure 1]c, suggesting an intramural hematoma, a pathognomonic feature of arterial dissection. Computed tomography (CT) angiography performed 10 h later after symptoms onset revealed a beaded appearance of the cervical V2 and V3 segments of the bilateral vertebral arteries [Figure 2]a and [Figure 2]b. These multifocal beaded-like stenoses existed not only in the left vertebral artery [[Figure 2]a, arrows], but also in the right vertebral artery [[Figure 2]b, arrowheads] along with an aneurysmal dilatation of the left VAD [[Figure 2]b, arrow]. No abnormalities were found on transthoracic echocardiography, Holter monitoring, or transcranial Doppler sonography with a saline agitation bubble study to evaluate potential embolic sources. Laboratory examinations, including C-reactive protein, rapid plasma reagin, anti-Sjögren's syndrome-related antigens A and B (anti-Ro, La), anti-neutrophil cytoplasmic antibody, anti-double-stranded DNA antibody, antiphospholipid antibody, serum IgG4, fluorescent antinuclear antibody, C3, C4, protein electrophoresis, and immunofixation electrophoresis levels were all within their normal limits.
The patient was treated with aspirin, nimodipine, high-dose statins, and anti-hypertensive medications for blood pressure control. The patient's symptoms of headache, limb dysmetria, and left-sided falling tendency resolved completely. However, her dizziness and nystagmus persisted for 2-month during the follow-up period. A follow-up CT angiogram performed approximately 3 months after the index stroke showed resolution of vasoconstriction and normalization of the vertebral arteries [Figure 2]c and [Figure 2]d.
Figure 1: (a) MRI of the brain showing a small hyperintense lesion in the left medulla on the diffusion-weighted image suggesting acute infarction. MRI T1 sequence revealed hyperintense lesion within the left vertebral artery (b), of which susceptibility was correspondingly observed on SWI MRI (c) compatible with an intramural hematoma in vertebral artery dissection. MRI: Magnetic resonance imaging, SWI: Susceptibility weighted image
Figure 2: (a and b) Initial CTA showing multifocal stenosis in the left (a, arrows) and the right (b, arrow heads) with a intracranial dissection in the left vertebral artery (b, arrow). (c and d) A follow-up CT angiogram performed at about 3 months after the index stroke showed resolution of vasoconstriction and normalization of vertebral arteries. CTA: Computed tomography angiography, CT: Computed tomography Discussion In this study, we report a patient with a VAD in the left intracranial V4 segment and concomitantly multifocal stenosis in the bilateral V2 and V3 segments of the extracranial vertebral arteries. These were completely resolved during the follow-up evaluation. Apart from the dissection in the left vertebral artery V4 segment, RCVS rather than VAD progression was deemed to cause this bilateral multifocal stenosis, based on the simultaneous involvement of bilateral vessels observed on the initial brain MRI performed 10 h after the stroke onset.
Although the underlying pathophysiological mechanism has not been elucidated, endothelial dysfunction and alterations in cerebral vascular tone leading to vasoconstriction might have resulted in the RCVS.[4],[5] Isolated RCVS is known to have an intracranial pathology. Extracranial RCVS is very rare.[6] We think that a similar mechanism as the development of the vasospasm in SAH could explain the pathophysiologic mechanism of the development of extracranial RCVS in intracranial VAD in our study. Vasospasm in SAH is believed to be produced by spasmogenic substances generated during the lysis of subarachnoid blood. Similarly, the direct and remote effect of spasmogenic substances produced by intramural hematoma in intracranial VAD could be a hypothetical mechanism for the development of the extracranial RCVS.
RCVS can occasionally be accompanied by cervical arterial dissection.[1],[2] Mawet et al. have reported that the association between cervical arterial dissection and RCVS is observed mostly in VAD, while carotid artery dissection constitutes most of the isolated cervical arterial dissection.[1],[7] However, even in this clinical setting, they reported that vasoconstrictions were exclusively limited to intracranial vessels with diffuse and bilateral involvement patterns.[1] In our study, RCVS associated with VAD was predominantly present in the bilateral extracranial vertebral arteries. Extracranial RCVS is a very unusual finding. Only a few cases of extracranial RCVS have been reported.[2],[3] The prevalence of symptomatic infarcts associated with cervical artery dissection and secondary intracranial RCVS was lower than isolated cervical artery dissection (5% vs. 79%) in a previous study.[1] Both cases including ours, which are the only cases of cervical artery dissection and concomitant extracranial RCVS, showed brain stem infarctions.[2]
ConclusionThe possibility of extracranial involvement in RCVS associated with VAD renders the diagnosis and/or discrimination of RCVS from VAD more challenging because of their similar vascular luminal morphology. We suggest that when there is too early diffuse multifocal stenosis for a dissection to evolve or when there is a bilateral involvement in the early stage, concomitant presence of RCVS should be considered even if its location is in an extracranial segment. Early recognition of co-existing RCVS can lead to an optimal treatment strategy because the pathophysiological mechanisms and clinical management of RCVS differ greatly from those of other vasculopathies including arterial dissection, vasculitis, and atherosclerotic stenosis. Knowledge of extracranial RCVS with VAD is crucial for timely diagnosis and treatment with a good outcome.
Ethical standards
The authors declare that this single case report has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki. Informed consents were obtained from the patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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