Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Reversible cerebral vasoconstriction syndrome (RCVS) is a group of disorders characterized by severe headaches and a narrowing of the blood vessels in the brain. RCVS is reversible and patients often recover within three months; the condition is frequently missed and is more common than most physicians realize. Serious complications, such as a stroke, can be associated with RCVS if not promptly diagnosed and treated.

RCVS happens when persistent contraction of the blood vessels (vasoconstriction) causes arteries to narrow. This reduces blood flow and oxygen delivery to the affected area of the body. When vasoconstriction affects the blood vessels of the brain, it is called cerebral vasoconstriction.

Symptoms

The most common symptom of RCVS is a sudden, intense headache, often referred to as a "thunder clap" headache. Other symptoms may include:

  • Seizures
  • Changes in vision
  • Difficulty understanding others when they are speaking
  • Difficulty speaking
  • Weakness on one side of the body

RCVS patients are at risk of experiencing a stroke or transient ischemic attack (TIA). Signs of a stroke include:

  • Sudden numbness or weakness of the face, arm or leg, especially on one side of the body
  • Sudden confusion
  • Sudden trouble speaking
  • Sudden trouble seeing in one or both eyes
  • Sudden trouble walking
  • Sudden dizziness, loss of balance or coordination
  • Sudden, severe headache with no known cause

The effects of an acute ischemic stroke may cause additional symptoms in women including:

  • Face, arm or leg pain
  • Hiccups or nausea
  • Chest pain or palpitations
  • Shortness of breath

If you notice one or more of these signs of stroke in another person or in yourself, do not wait to seek help. Call 9-1-1 immediately.


Causes and Risk Factors

The cause of RCVS is unknown. The condition is more commonly diagnosed in women between the ages of 20 to 50, and may be associated with the changes that happen in the body immediately after giving birth (postpartum) or changing birth control pills. A history of migraine is nearly always found in patients with RCVS.

Other risk factors associated with RCVS include:

  • Use of drugs
  • Use of alcohol, especially binge drinking
  • Use of certain prescription medications, such as anti-depressants
  • Use of nasal decongestants
  • Use of nicotine patches
  • Certain tumors
  • Elevated calcium levels in the blood (hypercalcemia)
  • Head trauma

Diagnosis

Diagnosis of RCVS usually begins with a physical exam and a review of the patient’s medical history and symptoms, as well as the findings of diagnostic tests. Given the association with migraine, however, not all patients with suspected RCVS will require advanced imaging tests.

Imaging tests, such as magnetic resonance imaging (MRI) and computed tomography (CT), are used to see the area affected by constricted blood vessels. These imaging tests look at the soft tissue and blood vessels within the body and can determine if the condition is associated with a stroke or other underlying issues.

Other tests look exclusively at the blood vessels within the body. An angiogram may also be used to view the arteries. A transcranial Doppler ultrasound is used to measure the blood that is flowing through the arteries at the base of the brain.

Blood and urine tests may also be used to examine how well the liver and kidneys are functioning.

Treatments

Treatment of RCVS depends on the severity of the condition. In some cases, the condition clears up without treatment. Due to the risk of stroke, however, it is important that patients seek and get immediate medical care. Fluids will be administered intravenously and migraine treatments such as aspirin or Depakote may be given.

In some cases, a calcium channel blocker may be used to relax the blood vessels and allow more blood to flow through. This medication has been shown to help ease the "thunder clap" headaches, but does not decrease the risk of stroke.

For patients who have experienced a stroke, the Stroke Program at Cedars-Sinai provides a multidisciplinary treatment approach through treatment plans tailored to each patient. Patient care is generally broken down into three categories: stroke prevention, treatment immediately after a stroke, and post-stroke rehabilitation.