The many faces of the reversible cerebral vasoconstriction syndrome (RCVS)

Abstract

The laboratory findings have received some recent modifications. Cerebrospinal fluid (CSF) may be normal or near-normal (protein concentration<100 mg/dL,<15 white blood cells/μL, normal glucose).3 There is a characteristic pattern of radiographic features alongside suggestive clinical manifestations, which lead to a diagnosis of RVCS.This syndrome is characterized:1-clinically by acute-onset and very severe headache, often thunderclap headache,ie headache peaking in one minute, and with or without acute neurological signs and symptoms, such as seizures, hemiparesis or encephalopathy. 2-radio logically by multifocal and segmental narrowing of multiple cerebral arteries corresponding to vasoconstriction that can affect the proximal, middle, and distal arterial segments and posterior predominant brain edema. The vasoconstriction is demonstrated by a characteristic diffuse “beaded” appearance identified onmagnetic resonance angiography (MRA), angio-CT(ACT) or catheter cerebral angiography.4,5 This syndrome is also defined by the complete resolution of the vasoconstriction shown by follow-up on MRA, CTA or catheter cerebral angiography within 12 weeks after onset, together with the total clinical normalization. Epidemiology RVCS affects patients of all ages, with a middle-aged female preponderance, between 20 and 50; the sex ratio varies among the series.The RVCS seems rare and perhaps under-recognized in children.6 Causes: There are various precipitants of RCVS including, sexual activity, exertion, pregnancy-related conditions (early puerperium, pre-eclampsia, and eclampsia; post-partum), sympathomimetic or vasoactive substances, including nasal decongestants, serotonine-reuptake inhibitors, catecholamine-secreting tumors and ergolinederivates, intravenous immunoglobulin, blood products, immune suppressants, and the use of illicit drugs(cannabis, cocaine) or comorbid auto-immune disorders. Many other putative precipitants have been reported. In 2/3 of patients, one or multiple precipitating factors are identified.