EXPLAIN IN DETAIL RCVS ( REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROME ) ?
A 5 DEFINITION
1 Reversible cerebral vasoconstriction syndrome(RCVS, sometimes called Call-Fleming syndrome) is a disease characterized by a weeks-long course of thunderclap headaches, sometimes focal neurologic signs, and occasionally seizures.
2 The average age of onset is 42, but RCVS has been observed in patients aged from 19 months to 70 years.
3 Children are rarely affected.
4 It is more common in females, with a female-to-male ratio of 2.4:1
CHARACTERISTICS / SIGNS AND SYMPTOMS / FEATURES
1 The key symptom of RCVS is recurrent thunderclap headaches, which over 95% of patients experience - In two-thirds of cases, it is the only symptom.
2 These headaches are typically bilateral, very severe and peak in intensity within a minute.
3 They may last from minutes to days, and may be accompaniedbynausea, photophobia, phonophobia or vomiting.
4 Some patients experience only one headache, but on average there are four attacks over a period of one to four weeks.
5 A milder, residual headache persists between severe attacks for half of patients.
6 1–17% of patients experience seizures.
7 8–43% of patients show neurologic problems, especially visual disturbances, but also hemiplegia, ataxia, dysarthria,aphasia, and numbness.
8 These neurologic issues typically disappear within minutes or a few hours; more persistent symptoms may indicate a stroke.
9 Posterior reversible encephalopathy syndrome is present in a small minority of patients.
10 This condition features the unique property that the patient’s cerebral arteries can spontaneously constrict and relax back and forth over a period of time without intervention and without clinical findings.
11 Vasospasm is common post subarachnoid hemorrhage and cerebral aneurysm, but in RCVS only 25% of patients have symptoms post subarachnoid hemorrhage vv imp
ETIOLOGY
1 The direct cause of the symptoms is believed to be either constriction or dilation of blood vessels in the brain.
2 The pathogenesis is not known definitively, and the condition is likely to result from multiple different disease processes.
3 Up to two-thirds of RCVS cases are associated with an underlying condition or exposure, particularlyvasoactive or recreational drug use, complications of pregnancy (eclampsia and pre-eclampsia), and the adjustment period following childbirth calledpuerperium.
4 Vasoactive drug use is found in about 50% of cases -
Illegal drugs associated with RCVS are:
1 Marijuana
2 Cocaine
3 Ecstasy
4 Amphetamine derivatives
5 Lysergic acid diethylamide (LSD)
5 Implicated drugs include selective serotonin reuptake inhibitors, weight-loss pills such as Hydroxycut, alpha-sympathomimeticdecongestants, acute migraine medications,pseudoephedrine, epinephrine, cocaine, andcannabis, among many others.
6 It sometimes follows blood transfusions, certain surgical procedures, swimming, bathing, high altitudeexperiences, sexual activity, exercise, or coughing.
7 Symptoms can take days or a few months to manifest after a trigger.
8 Following a study and publication in 2007, it is also thought SSRIs, uncontrolled hypertension, endocrine abnormality, and neurosurgical trauma are indicated to potentially cause vasospasm ( vv imp )
9 Prescription medications associated with RCVS include the following - vv imp
1 Antidepressants: Selective serotonin reuptake inhibitors (SSRIs) such as Prozac®, Paxil®, and Zoloft®
2 Medications to treat migraines: Triptans (Imitrex®, Maxalt®), Isometheptine (Amidrine®, Midrin®), and Ergotamines (Migergot®, Ergomar®, Cagergot®)
3 Immunosuppressants: Cyclophosphamide (Cytoxan®) and Tacrolimus (FK-506®)
4 Drugs to prevent bleeding after childbirthAnti-Parkinson’s medications: Bromocriptine and Lisuride (Dopergin®, Proclacam®,and Revanil®)
10 Others factors related to RCVS can include blood and intravenous immunoglobulin (IVIG) transfusions as well as vasoactive secreting tumors. These tumors include phaeochromocytoma, bronchial carcinoid, and glomus tumors.
DIAGNOSIS
1 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.
2 This may involve aCT scan, lumbar puncture, MRI, and other tests.
3 Posterior reversible encephalopathy syndrome has a similar presentation, and is found in 10–38% of RCVS patients.
4 RCVS is diagnosed by detecting diffuse reversible cerebral vasoconstriction.
5 Catheterangiography is ideal, but computed tomography angiography and magnetic resonance angiography can identify about 70% of cases.
6 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.
RADIOLOGICAL FEATURES
1 angiography (CTA/MRA/DSA) demonstrates multifocal narrowings in the circle of Willis and its branches; post-stenotic segments show dilatation giving classical beaded appearance/sausage-shaped arteries.
PROGNOSIS
1 All symptoms normally resolve within three weeks, and may only last days.
2 Permanent deficits are seen in a minority of patients, ranging from under 10% to 20% in various studies.
3 Less than 5% of patients experience progressive vasoconstriction, which can lead to stroke, progressive cerebral edema, or even death.
4 Severe complications appear to be more common in postpartum mothers.
TREATMENT
1 As of 2014, no treatment strategy has yet been investigated in a randomized clinical trial.
2 Verapamil, nimodipine, and other calcium channel blockers may help reduce the intensity and frequency of the headaches.
3 A clinician may recommend rest and the avoidance of activities or vasoactive drugs which trigger symptoms .
4 Analgesics and anticonvulsants can help manage pain and seizures, respectively