Explain in detail rcvs ( reversible cerebral vasoconstriction syndrome )?

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