Reversible cerebral vasoconstriction syndrome
|Reversible cerebral vasoconstriction syndrome|
|Other names: Call-Fleming syndrome, postpartum cerebral angiopathy, benign angiopathy of the central nervous system, migrainous vasospasm, migraine angiitis|
|Significant beading of all intracranial arteries, best demonstrated in the anterior cerebral arties due to RCVS.|
|Symptoms||Recurrent and severe headaches, vomiting, sensivity to light, confusion, focal neurologic signs, seizures|
|Complications||Subarachnoid hemorrhage, stroke|
|Usual onset||20 to 50 years old|
|Risk factors||Childbirth, vasoactive drugs, complications of pregnancy|
|Diagnostic method||Medical imaging|
|Differential diagnosis||Subarachnoid hemorrhage due to an aneurysm, cerebral artery dissection, cerebral venous sinus thrombosis, ischemic stroke, pituitary apoplexy, cerebral vasculitis|
|Treatment||Calcium channel blockers|
Reversible cerebral vasoconstriction syndrome (RCVS) is a disease characterized by many areas of constriction and dilation of arteries around the brain. Symptoms typically include recurrent and severe headaches of sudden onset (thunderclap headaches). Other symptoms may include vomiting, sensivity to light, confusion, focal neurologic signs, and seizures. Complications may include subarachnoid hemorrhage and stroke.
Risk factors include childbirth, vasoactive drugs, and complications of pregnancy. Drugs that have been implicated include cocaine, sumatriptan, diet pills, SSRIs, and pseudoephedrine. Diagnosis is generally by medical imaging. Other conditions that may present similarly include subarachnoid hemorrhage due to an aneurysm, cerebral artery dissection, cerebral venous sinus thrombosis, ischemic stroke, pituitary apoplexy, and cerebral vasculitis.
Calcium channel blockers, such as nimodipine, have been used for treatment. For the vast majority, all symptoms disappear on their own within three weeks. Deficits persist in a minority, with severe complications or death being very rare.
While how often it occurs is unknown, it is believed to be relatively common. Those affected are most often 20 to 50 years old. Females are affected more often than males. The condition was first described in the 1960s; however, the current name did not come into use until 2007.
Signs and symptoms
The key symptom of RCVS is recurrent thunderclap headaches, which occurs in over 95%. In two-thirds of cases, it is the only symptom. These headaches are typically bilateral, very severe and peak in intensity within a minute. They may last from minutes to days, and may be accompanied by nausea, photophobia, phonophobia or vomiting. Some people experience only one headache, but on average there are four attacks over a period of one to four weeks. A milder, residual headache persists between severe attacks for half of people.
About 1–17% of people experience seizures; 8–43% of people show neurologic problems, especially visual disturbances, but also hemiplegia, ataxia, dysarthria, aphasia, and numbness. These neurologic issues typically disappear within minutes or a few hours; more persistent symptoms may indicate a stroke. Posterior reversible encephalopathy syndrome is present in a small minority of people.
This condition features the unique property that the person's cerebral arteries can spontaneously constrict and relax back and forth over a period of time without intervention and without clinical findings. Vasospasm is common post subarachnoid hemorrhage and cerebral aneurysm, but in RCVS only 25% of people have symptoms post subarachnoid hemorrhage.
The direct cause of the symptoms is believed to be either constriction or dilation of blood vessels in the brain. The pathogenesis is not known definitively, and the condition is likely to result from multiple different disease processes.
Up to two-thirds of RCVS cases are associated with an underlying condition or exposure, particularly vasoactive or recreational drug use, complications of pregnancy (eclampsia and pre-eclampsia), and the adjustment period following childbirth called puerperium. Vasoactive drug use is found in about 50% of cases. Implicated drugs include selective serotonin reuptake inhibitors, weight-loss pills such as Hydroxycut, alpha-sympathomimetic decongestants, acute migraine medications, pseudoephedrine, epinephrine, cocaine, and cannabis, among many others. It sometimes follows blood transfusions, certain surgical procedures, swimming, bathing, high altitude experiences, sexual activity, exercise, or coughing. Symptoms can take days or a few months to manifest after a trigger.
The clinician should first rule out conditions with similar symptoms, such as subarachnoid hemorrhage, ischemic stroke, pituitary apoplexy, cerebral artery dissection, meningitis, and spontaneous cerebrospinal fluid leak. This may involve a CT scan, lumbar puncture, MRI, and other tests. Posterior reversible encephalopathy syndrome has a similar presentation, and is found in 10–38% of people.
RCVS is diagnosed by detecting diffuse reversible cerebral vasoconstriction. Catheter angiography is ideal, but computed tomography angiography and magnetic resonance angiography can identify about 70% of cases. Multiple angiographies may be necessary. Because other diseases (such as atherosclerosis) have similar angiographic presentations, it can only be conclusively diagnosed if vasoconstriction resolves within 12 weeks.
All symptoms normally resolve within three weeks, and may only last days. Permanent deficits are seen in a minority of people, ranging from under 10% to 20% in various studies. Less than 5% of people experience progressive vasoconstriction, which can lead to stroke, progressive cerebral edema, or even death. Severe complications appear to be more common in postpartum mothers.
The incidence of RCVS is unknown, but it is believed to be "not uncommon", and likely under-diagnosed. One small, possibly biased study found that the condition was eventually diagnosed in 45% of outpatients with sudden headache, and 46% of outpatients with thunderclap headache.
The average age of onset is 42, but RCVS has been observed in those aged from 19 months to 70 years. Children are rarely affected. It is more common in females, with a female-to-male ratio of 2.4:1.
As of 2014, no treatment strategy has yet been investigated in a randomized clinical trial. Verapamil, nimodipine, and other calcium channel blockers may help reduce the intensity and frequency of the headaches. A clinician may recommend rest and the avoidance of activities or vasoactive drugs which trigger symptoms (see § Causes). Analgesics and anticonvulsants can help manage pain and seizures, respectively.
Case studies of the condition first appeared in the 1960s, but it was not then recognized as a distinct entity. In 1983, French researchers published a case series of 11 people, terming the condition acute benign cerebral angiopathy. Gregory Call and Marie Fleming were the first two authors of a report in which doctors from Massachusetts General Hospital, led by C. Miller Fisher, described 4 people, alongside 12 previous case studies, with the characteristic symptoms and abnormal cerebral angiogram findings. The name Call-Fleming syndrome refers to these researchers.
A 2007 review by Leonard Calabrese and colleagues proposed the name reversible cerebral vasoconstriction syndrome, which has since gained widespread acceptance. This name merges various conditions that were previously treated as distinct entities, including Call-Fleming syndrome, postpartum angiopathy, and drug-induced angiopathy. Other names may still be used for particular forms of the condition.
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