What is reversible cerebral vasoconstriction syndrome (RCVS)?

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare condition that occurs as the result of a sudden constriction (tightening) of the vessels that supply blood to the brain. The main symptom of RCVS is sudden, severe, and disabling headaches that are sometimes called “thunderclap” headaches. Strokes or a bleeding into the brain may or may not be present.

RCVS can be reversed. Outcomes for RCVS patients range from full recovery in most patients to permanent brain damage in other patients. RCVS is most common among females from the ages of 20 to 50.

Is reversible cerebral vasoconstriction syndrome (RCVS) dangerous?

Most patients with RCVS recover completely. A minority of patients have neurological problems. RCVS does not usually come back.

What is the major condition that resembles reversible cerebral vasoconstriction syndrome (RCVS)?

Central nervous system (CNS) vasculitis can be very similar to RCVS. CNS Vasculitis is a condition that results in inflammation of blood vessel walls of the brain or spine. (The brain and the spine make up the central nervous system). CNS vasculitis often occurs in the following situations:

• It appears along with other autoimmune diseases such as systemic lupus erythematosus, dermatomyositis, and rheumatoid arthritis (in rare cases).
• It may come along with a viral or bacterial infection.
• It may come along with systemic (affecting the whole body) vasculitic disorders (granulomatosis with polyangiitis (GPA), microscopic polyangiitis, Behçet’s syndrome).
• It may appear on its own; in such cases it is referred to as primary angiitis of the CNS (PACNS).

Healthcare providers need to be aware of RCVS and tell the difference between RCVS and CNS vasculitis as soon as possible. The two disorders differ in treatment and outlook. Treatment of RCVS does not require immunosuppressive drugs, but CNS vasculitis does.

What is the cause of reversible cerebral vasoconstriction syndrome (RCVS)?

A disturbance in the control of the smooth muscle tone inside of the brain’s blood vessel walls is thought to cause RCVS. There is no evidence of inflammation or changes within the structure of the brain’s blood vessels or tissue among persons with RCVS. There is no known cause for the change in the tone of blood vessels. The change is often spontaneous.

Some possible external factors related to RCVS may include the use of prescription, over the counter, or illegal drugs that can cause constriction of the arteries. RCVS also may be linked to internal factors such as tumors, which secrete substances that, in turn, constrict blood vessels.

Prescription medications associated with RCVS include the following:

• Antidepressants: Selective serotonin reuptake inhibitors (SSRIs) such as Prozac®, Paxil®, and Zoloft®
• Medications to treat migraines: triptans (Imitrex®, Maxalt®), isometheptene (Amidrine®, Midrin®), and ergotamines (Migergot®, Ergomar®, Cafergot®)
• Immunosuppressants: cyclophosphamide (Cytoxan®) and tacrolimus (FK-506®)
• Drugs to prevent bleeding after childbirth
• Anti-Parkinson’s medications: bromocriptine and lisuride (Dopergin®, Proclacam®,and Revanil®)

Common over-the-counter drugs and supplements that can cause constriction of cerebral arteries include the following:

• Nasal decongestants (pseudoephedrine, ephedrine, phenylpropanolamine)
• Nicotine patches
• Caffeine-containing energy drinks
• Ginseng

Illegal drugs associated with RCVS are:

• Marijuana
• Cocaine
• Ecstasy
• Amphetamine derivatives
• Lysergic acid diethylamide (LSD)

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.

What are the symptoms of reversible cerebral vasoconstriction syndrome (RCVS)?

The severe and sudden onset of headaches is present in all patients. Other symptoms may include:

• Strokes or transient ischemic attacks (TIAs or “mini-strokes”)
• Weakness
• Problems with eyesight
• Seizures

How is reversible cerebral vasoconstriction syndrome (RCVS) diagnosed?

The diagnosis of RCVS is based on a person's medical history, symptoms, a complete physical exam, and the results of vascular brain imaging. Such imaging may be in the form of an angiogram, MRA, or CTA scan that can show spasms in blood vessels that are narrowed.

Cerebrospinal fluid testing can help to rule out other conditions that appear to be similar to RCVS. It is important to repeat the vascular imaging to ensure that the spasm that resulted in the diagnosis can be confirmed.

How is reversible cerebral vasoconstriction syndrome (RCVS) treated?

There is no known cure for RCVS. If a drug has been associated with RCVS, the patient should talk to his or her doctor about decreasing the dose or stopping the use of the drug. The use of calcium channel blockers such as Cardizem® and nimodipine can reduce headaches. In some cases, the patient is given intravenous magnesium. Rest is very important in the treatment of RCVS.