A condition in which a person experiences sudden headaches and other disruptive brain symptoms is called reversible cerebral vasoconstrictive syndrome (RCVS). The symptoms of this disorder are usually similar to other medical emergencies, including brain bleeding and strokes. People who develop RCVS require immediate treatment. Otherwise, it may lead to permanent damage to the brain tissue and even death.
In some cases, these painful headaches that occur suddenly are called thunderclap headaches. This condition often occurs when a blood vessel suddenly spasms or constricts (narrows) in the brain. However, if you notice that a person experiences sudden, severe headaches, immediately call 911 or go to the nearest emergency room.
Moreover, this condition is also known as Call-Fleming syndrome. The name of this disorder comes from two physicians (George Call and Marie Fleming) who first diagnosed RCVS in 1988.
Symptoms
While the primary symptom of this disorder is thunderclap headaches, people with RCVS may also experience other symptoms. Check below some examples:
- Photophobia (increased sensitivity to light)
- Nausea
- Vomiting
- Muscle weakness
- Vision changes (such as trouble seeing)
- Confusion
- Seizures
- Phonophobia (sound sensitivity)
- Aphasia (difficulty understanding other people when they talk or trouble speaking)
In addition, RCVS may sometimes cause subarachnoid hemorrhage (SAH). This is a type of bleeding that occurs between the brain and skull (also called a brain bleed). SAH is also a medical emergency. Check below some symptoms that people who develop SAH may experience:
- Personality and mood changes (such as irritability)
- Numbness that negatively affects a part of the body
- Neck stiffness
- Muscle aches in the shoulder and neck
If you experience any of the previous symptoms, immediately seek medical care.
Causes
In normal circumstances, blood vessels have a lining of smooth muscle inside them that allows them to widen and narrow to limit or increase blood flow. RCVS often occurs when this muscle layer tightens and spasms that limiting blood flow (also called vasospasm).
In other words, the blood flows through blood vessels similarly to liquid through a straw. If you try to crimp or squeeze it, you will notice that liquids cannot flow easily through that straw. In general, the brain has a very high demand for blood flow, and even a minor reduction in blood flow may cause functional problems.
RCVS occurs due to multiple factors and causes. For example, pregnancy and pregnancy-related disorders, medicines and nonmedical drug use, diseases, and certain medical procedures.
Pregnancy and Pregnancy-related Disorders
Pregnancy itself is one of the most common factors that causes RCVS. About 50% of all diagnosed cases occur after childbirth, especially in the first 6 weeks. Doctors think it is related to hormonal changes that occur during pregnancy. RCVS may also occur even if you do not have a pregnancy-related disorder, including preeclampsia, eclampsia, or HELLP syndrome.
Medical and Nonmedical Drug and Substance Use
In general, drugs affect the circulatory system and may contribute to or even cause RCVS. Check below some examples:
- Immunosuppressants
- Migraine medicines, including ergot-based drugs, triptans, and others
- Medications used to treat Parkinson’s disease
- Stimulants (such as amphetamines, cocaine, and pseudoephedrine)
- Marijuana (cannabis)
- Caffeine and energy drinks
- Tobacco products
- Antidepressants, including selective serotonin reuptake inhibitors (SSRIs)
- Psychedelics (hallucinogens), including MDMA or LSD
Diseases
The following condition may negatively affect the way blood flows inside the brain. For example:
- Migraines – Generally, people with this disorder are more prone to develop RCVS compared to people without it.
- Hypercalcemia (high Calcium levels) – If you have an increased level of Calcium in the blood, it may disrupt how the muscles of the blood vessels work.
- Posterior reversible encephalopathy syndrome – This is a condition similar to RCVS that may occur at the same time. However, it mostly affects the back of the brain, while RCVS impacts the whole brain.
- Hormone-related factors and disorders – According to some researchers, sex hormones (especially Estrogen) contribute to RCVS. That’s why this condition mostly occurs in females.
- Genetics – There are some inherited disorders that significantly increase the risk of developing RCVS. For example, pheochromocytoma causes either benign or malignant tumors that produce and release extra adrenaline into the bloodstream. Another one that causes a buildup of some chemicals that the body naturally produces is called porphyria.
What Are The Potential Complications of RCVS?
Previously, healthcare providers thought this disorder was not dangerous. However, some recent studies have shown that about 30% of people with RCVS have mild symptoms, and 8% may develop more serious health problems. Check below some complications that people with RCVS may experience:
- Ischemic stroke
- SAH
- Leukoencephalopathy (white matter disease)
- Seizures
- Intracerebral hemorrhage (ICH)
- Cerebral edema (swelling of the brain)
Furthermore, sudden, severe headaches may recur within a week, or sometimes, people develop prolonged headaches, but they are often mild.
How to Prevent RCVS?
In general, it cannot always be prevented because this disorder often happens unpredictably. Moreover, healthcare providers do not fully understand why it happens. However, you can take some steps to reduce the risk. For example, never administer prescription, over-the-counter (OTC), or recreational drugs without your doctor’s approval, quit smoking, limit or avoid alcoholic drinks, and others. For more details, discuss it with your physician.
Diagnosis
Usually, the diagnosis begins with neurological and physical examinations to check for irregularities linked to the disease. Physicians may also ask some questions about the symptoms and medical history. However, to confirm the condition, they may perform some tests, including blood tests to check for clotting problems, infections, electrolyte imbalances, and others. The best way to confirm RCVS is with imaging tests. For example:
- CT (computed tomography) scans, especially CT angiograms
- MRI (magnetic resonance imaging) scans, especially MRA (magnetic resonance angiograms)
- Transcranial Doppler ultrasound
Previous tests are used to see how the blood flows through the blood vessels that supply the brain with oxygen-rich blood. They can identify if the brain does not receive enough blood.
Treatment
In general, this condition can be treated by stopping or removing the cause of vasospasm. Check below some treatments often recommended by doctors for people with RCVS:
- Calcium channel blockers (CCBs) – This is the most common group of medicines used to treat vasospasm. They help prevent cells from using Calcium and keep the smooth muscle lining of the blood vessels. As a result, these blood vessels widen, which increases blood flow.
- Endovascular procedure – This procedure helps treat a narrowed blood vessel from the inside. During this procedure, physicians use a long and flexible tube (catheter) that is inserted into a major blood vessel and then guided to the narrowed part that limits blood flow.
- Angioplasty – This treatment also uses a catheter to inflate a balloon that helps widen and keep open an artery.
Frequently Asked Questions
How long does RCVS last?
The recovery time depends on several factors. These include how fast you get treatment, your response to treatment, the severity of the symptoms, existing health problems, and others. Consult with your physician for more details.
What is the outlook for RCVS?
In most cases, people successfully recover with treatment without any long-lasting symptoms. However, 10% of people may experience lasting mild symptoms. Less commonly, RCVS causes life-threatening complications (such as strokes). Without immediate treatment, it may lead to permanent brain damage or even death.
What can trigger RCVS?
There are some factors that could trigger this disorder. For example, pregnancy and postpartum disorders, some medical procedures, recreational drug use, and some prescription medicines. Ask your healthcare provider if you have additional questions.
