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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

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Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

T.R. Miller, R. Shivashankar, M. Mossa-Basha and D. Gandhi
American Journal of Neuroradiology August 2015, 36 (8) 1392-1399; DOI: https://doi.org/10.3174/ajnr.A4214
T.R. Miller
aFrom the Department of Diagnostic Radiology (T.R.M., R.S., D.G.), Section of Neuroradiology, University of Maryland Medical Center, Baltimore, Maryland
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R. Shivashankar
aFrom the Department of Diagnostic Radiology (T.R.M., R.S., D.G.), Section of Neuroradiology, University of Maryland Medical Center, Baltimore, Maryland
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M. Mossa-Basha
bDepartment of Diagnostic Radiology (M.M.-B.), Section of Neuroradiology, University of Washington, Seattle, Washington.
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D. Gandhi
aFrom the Department of Diagnostic Radiology (T.R.M., R.S., D.G.), Section of Neuroradiology, University of Maryland Medical Center, Baltimore, Maryland
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  • Fig 1.
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    Fig 1.

    A 47-year-old woman with the sudden onset of severe headache. Initial noncontrast head CT (A) demonstrates trace sulcal subarachnoid hemorrhage (white arrow) near the vertex. CT angiography performed at the same time (B) is interpreted as having unremarkable findings. Conventional angiography (C) demonstrates mild diffuse irregularity with multifocal narrowings throughout the cerebral vasculature with a beaded appearance, most pronounced in distal right middle cerebral artery cortical branches (black arrow). Findings are most consistent with RCVS. Follow-up catheter angiogram performed 1 month later (D) demonstrates complete resolution of cerebral vasoconstriction (black arrow).

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    Fig 2.

    A 42-year-old woman who presented with altered mental status and lethargy. FLAIR imaging (A) demonstrates signal hyperintensity involving the cortex and underlying subcortical white matter in the parietal and occipital lobes (white arrows), consistent with PRES. There is no evidence of associated diffusion restriction. Trace sulcal subarachnoid hemorrhage was also noted overlying the right frontal lobe (not shown). Note subtle irregularity and multifocal narrowings involving distal cortical branches of the bilateral middle and anterior cerebral arteries (black arrows) on cerebral angiography (B), suggestive of RCVS. The patient made a full recovery, with complete resolution of cerebral areas of abnormal FLAIR hyperintensity (C) and cerebral vasoconstriction (not shown).

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    Fig 3.

    A 19-year-old man with a 2-day history of recurrent headaches and prior marijuana use. Noncontrast CT was negative for acute hemorrhage (not shown). Conventional angiography (A) reveals multifocal areas of moderate narrowing and irregularity involving the cerebral vasculature (white arrows, A). These areas resolved following intra-arterial administration of verapamil (white arrows, B). Clinical course and imaging findings are consistent with RCVS.

Tables

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    Table 1:

    Prior terms for RCVS

    Prior Terms
    Migrainous vasospasm
    Benign angiopathy of the central nervous system
    Postpartum angiopathy
    Thunderclap headache with reversible vasospasm
    Sexual headache
    Drug-induced angiopathy
    Call-Fleming syndrome
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    Table 2:

    Diagnostic criteria for RCVS

    Criteria
    Severe, acute headaches, with or without additional neurologic signs or symptoms
    Uniphasic disease course with no new symptoms after 1 month of onset
    No evidence for aneurysmal SAH
    Normal or near-normal findings on CSF analysis (protein level, <80 mg/dL; leukocyte level, <10/mm3; normal glucose level)
    Multifocal segmental cerebral artery vasoconstriction demonstrated on either catheter angiography or indirectly on CTA/MRA
    Reversibility of angiographic abnormalities within 12 weeks after onset. If death occurs before the follow-up studies are completed, postmortem rules out such conditions as vasculitis, intracranial atherosclerosis, and aneurysmal SAH, which can also manifest with headache and stroke
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    Table 3:

    Potential triggers of RCVS

    Triggers of Secondary RCVS
    Vasoactive medications
        Sympathomimetic drugs, bromocriptine, ergotamine, pseudoephedrine, selective serotonin-uptake inhibitors, interferon, triptans, diet pills, nonsteroidal anti-inflammatory drugs
    Vasoactive recreational drugs
        Alcohol, amphetamines, cannabis, cocaine, ecstasy, nicotine
    Pregnancy and postpartum states
    Blood products
        Blood transfusions, erythropoietin, intravenous immunoglobulin
    Headache disorders
        Migraines
    Tumors
        Pheochromocytoma
        Paraganglioma
    Trauma
    Carotid dissection, unruptured cerebral aneurysm
    Head and neck surgery
    Various medical conditions
        Hemolysis, elevated liver enzymes, low platelets
        Antiphospholipid antibody syndrome
        Thrombotic thrombocytopenic purpura
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American Journal of Neuroradiology: 36 (8)
American Journal of Neuroradiology
Vol. 36, Issue 8
1 Aug 2015
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Cite this article
T.R. Miller, R. Shivashankar, M. Mossa-Basha, D. Gandhi
Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course
American Journal of Neuroradiology Aug 2015, 36 (8) 1392-1399; DOI: 10.3174/ajnr.A4214

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Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course
T.R. Miller, R. Shivashankar, M. Mossa-Basha, D. Gandhi
American Journal of Neuroradiology Aug 2015, 36 (8) 1392-1399; DOI: 10.3174/ajnr.A4214
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