Reversible Cerebral Vasoconstriction Syndromes

What is Reversible Cerebral Vasoconstriction Syndromes?

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a condition that causes severe and abrupt headaches that last for days to weeks. This occurs due to tightening of the blood vessels in the brain. While RCVS usually goes away on its own and doesn’t cause lasting harm, it can sometimes lead to serious conditions. These include ischemic stroke (a condition where blood flow to the brain is blocked), subarachnoid or intracerebral hemorrhage (bleeding into the brain or the space surrounding the brain), and posterior reversible encephalopathy syndrome (a brain disorder that can cause seizures, headaches, and vision changes).

RCVS is now recognized as a disorder that affects the brain’s blood vessels. It’s mainly characterized by severe headaches that occur suddenly and are often referred to as “thunderclap headaches”. These are intense headaches that happen recurrently, and their occurrence is indicative of RCVS. Common triggers can be certain blood vessel-constricting medications and many chemotherapy drugs. RCVS is usually temporary and goes away on its own.

RCVS and another syndrome known as Posterior Reversible Encephalopathy Syndrome (PRES) are both linked to abnormal regulation of brain blood vessels. They have similar clinical and imaging features, including headaches, visual disturbances, seizures, and confusion. To manage these conditions, potential triggers have to be eliminated, headaches and seizures need to be treated symptomatically, and moderate blood pressure should be maintained.

RCVS, which is likely caused by abnormalities in the lining of blood vessels and the tone of cerebral vessels, can be seen on a type of brain imaging known as cerebral angiography. Here, the constriction of blood vessels gives a classic “sausage on a string” look. However, imaging techniques that look at the brain’s structure often do not show any abnormalities, especially in the early stages.

Before RCVS was more understood, many patients were wrongly diagnosed with primary angiitis of the central nervous system (PACNS) (a rare inflammation of the brain and spinal cord’s blood vessels), which led to unnecessary risks associated with brain biopsies and long-term immunosuppression. More accurate understanding and diagnostic methods, such as the RCVS2 score, have greatly reduced this confusion.

What Causes Reversible Cerebral Vasoconstriction Syndromes?

Reversible cerebral vasoconstriction syndrome (RCVS) is a condition involving temporary but severe narrowing of the brain’s blood vessels. The link between recurring thunderclap headaches (TCHs) – sudden and severe headaches – and the narrowing of brain blood vessels in RCVS is complex and isn’t completely understood. Some theories suggest that connections between brain blood vessels and the lower part of the brain, involving certain pathways, could be part of the cause.

Having high blood pressure may add to damage in the brain’s blood-brain barrier – a natural defence that stops harmful substances from reaching the brain – which is often seen in situations of brain regulation disorder.

Many different triggers can lead to RCVS, most of them linked to an increase in the body’s ‘fight or flight’ response. Major triggers are common types of severe headaches, especially those linked with physical exertion. Women in the late stages of pregnancy or just after giving birth are more susceptible to RCVS, as are individuals with high blood pressure conditions during pregnancy like eclampsia and pre-eclampsia. Numerous legal and illegal drugs have also been linked with RCVS – from cold and cough medications and antidepressants to cocaine and cannabis. Even everyday substances like coffee and licorice can set off RCVS.

Other triggers may include tumors such as pheochromocytoma and paraganglioma, physical trauma, surgery involving the head and neck, and injury to the carotid artery. Certain medical conditions, such as an immune condition causing abnormal blood clotting and a rare blood disorder can contribute to RCVS. The start of RCVS can range from a few days to several months after exposure to these triggers.

There aren’t any definitively identified genetic risk factors for RCVS. However, occasionally, RCVS has been seen in patients with genetic diseases, like adult-onset Leigh syndrome, a rare genetic disorder affecting the central nervous system. One study reported that a certain genetic variation might have an impact on RCVS, with results showing potentially higher narrowing of arteries in certain individuals.

Risk Factors and Frequency for Reversible Cerebral Vasoconstriction Syndromes

RCVS, or Reversible Cerebral Vasoconstriction Syndrome, is a rare brain condition that has been gaining more attention in the medical community over the past two decades. The number of published studies on RCVS has increased from only 3 in 2003, to 80 by 2023. However, because there haven’t been any large-scale studies, we don’t fully understand how common this condition is. This potential increase could be due to doctors becoming more aware of RCVS and using imaging technology more often. A 2019 study in the Stroke journal suggested that the number of cases could vary greatly, from 7% to 54%.

RCVS is more common in adult women, with anywhere from 2 to 10 times as many cases in women compared to men. Interestingly, in children, boys get RCVS more often than girls. People of all ages can get RCVS, from newborns all the way up to people in their early 80s. However, most people start experiencing symptoms in their 50s.

RCVS is a condition that affects the brain.
The number of studies on RCVS increased from 3 in 2003 to 80 in 2023.
Doctors’ increased awareness and the use of imaging technology might be reasons why more cases are being reported.
The occurrence of RCVS could range between 7% to 54%, but it varies significantly.
It’s more common in adult women – between 2 to 10 times more than in men.
RCVS affects boys more often than girls in children.
People of all ages can get RCVS, but it usually starts in the 50s.
RCVS has been known by many other names in the past, including benign angiopathy of the CNS, Call-Fleming syndrome, and CNS pseudovasculitis.

Signs and Symptoms of Reversible Cerebral Vasoconstriction Syndromes

Reversible cerebral vasoconstriction syndrome (RCVS) often shows up as a sudden, severe headache, sometimes referred to as a thunderclap headache (TCH). These headaches typically reach their worst within a minute and tend to repeat, often weekly, for up to a month. The intensity and frequency of these headaches decrease over that period. Doctors might suspect RCVS in anyone with a TCH, and they’ll ask detailed questions about the patient’s headache history.

Some RCVS patients may experience seizures or neurological issues within the first 10 days of their recurring headaches. Symptoms pointing to possible brain injury, like difficulty with speech or weak muscles on one side of the body, warrant attention. Visual symptoms like blurry vision, temporary blindness, and blind spots are also common.

Signs of broad neurological trouble might include seizures and encephalopathy, a general term for brain disease that alters brain function. Seizures in cases of RCVS are typically characterized by muscle stiffening and violent shaking but are usually self-limiting. Seizures are less common in RCVS than in another similar condition, PRES (Posterior reversible encephalopathy syndrome).

Doctors will ask about possible triggers, like recent surgery (especially brain surgery), childbirth, medications, and recreational substances. Certain medications and substances – legal and illegal – have been linked with RCVS. Even though no direct genetic causes have been identified for RCVS, doctors will still ask about family medical history, as RCVS can be associated with inheritable conditions like pheochromocytoma and porphyria.

A 2019 study introduced a rating scale called the RCVS2 score. It uses five factors to assess the likelihood of RCVS diagnosis, with a maximum score of 10:

The presence of TCH
Involvement of arteries inside the skull
Being female
Identifying a trigger that constricts blood vessels
The presence of bleeding around the brain

Just having a TCH gives a score of 5. A score of 5 or higher indicated a 90% chance that the diagnosis will be RCVS, according to the study.

Testing for Reversible Cerebral Vasoconstriction Syndromes

If you’re being evaluated for a thunderclap headache, which is a severe and sudden headache, your doctor will take a detailed medical history. They’ll want to know about anything that might have triggered the headache, if you’ve used certain medications or substances recently, and if you’ve had any recent confusion or seizures. You’ll also receive a thorough neurological exam, specifically focusing on your level of consciousness, your mental sharpness, any visual symptoms, and if you have signs of a stroke like difficulty speaking, neglecting one side, weakness on one side of your body, and loss of sensation on one side.

Thunderclap headaches can be connected to reversible cerebral vasoconstriction syndrome (RCVS), which is a condition affecting blood vessels in the brain. Interestingly, about 70% of RCVS patients have normal brain imaging results when they first present symptoms. However, within the next two weeks, about 70% of hospitalized patients are likely to develop brain hemorrhage (bleeding in the brain), ischemic stroke (stroke caused by a blockage), or vasogenic edema (swelling in the brain).

To diagnose the cause of a thunderclap headache, your healthcare provider will conduct some tests, and the type of tests can be guided by the nature of the headache. If the thunderclap headache is recurrent, or happening repeatedly, the RCVS2 score can be used to assess the likelihood of RCVS. If the score suggests RCVS is probable, doctors might not need to do invasive diagnostic tests, such as a lumbar puncture (a procedure to collect and look at the fluid surrounding the brain and spinal cord) or brain biopsy (a procedure to remove a small sample of brain tissue).

In cases of a single thunderclap headache, your doctor will look to rule out life-threatening conditions such as aneurysmal subarachnoid hemorrhage (a type of stroke caused by bleeding in the space around the brain), pituitary apoplexy (bleeding or impaired blood supply to the pituitary gland), cervical artery dissection (tear in the wall of the artery in the neck), meningitis (inflammation of the membranes surrounding the brain), and cerebral venous sinus thrombosis (blood clot in the brain’s venous sinuses). They will carry out urgent brain and blood vessel imaging. If these images don’t show a subarachnoid hemorrhage, a lumbar puncture can be performed to confirm it or to rule out meningitis.

In recurrent thunderclap headache cases, brain imaging is still necessary, primarily to check for possible effects of RCVS, such as bleeding areas or ischemic stroke.

When looking at affected arteries in RCVS with angiography (an imaging test that uses x-rays and a special dye to see inside the arteries), it’s common to see a “sausage on a string” appearance. This is due to smoothly tapered narrowing alternating with dilation of the blood vessels. Other tests like transcranial ultrasound may also reveal elevated blood flow.

Treatment Options for Reversible Cerebral Vasoconstriction Syndromes

The treatment for Reversible Cerebral Vasoconstriction Syndrome (RCVS), a condition that involves a temporary narrowing of blood vessels in the brain, is largely focused on recognizing it as a condition that usually gets better on its own. The aim is to avoid a misdiagnosis that may lead to unnecessary invasive tests. The preferred approach is to treat the symptoms over introducing general treatments. If certain triggers of the condition are identified, it’s crucial to remove these, hopefully until the patient no longer experiences symptoms and test results return to normal.

For this condition, the use of long-term medication for seizures is not usually necessary unless there’s irreversible brain injury. When ut comes to managing symptoms, it’s important to treat headaches and high blood sugar levels. Both can potentially worsen patient outcomes. Patients undergoing glucocorticoid therapy, a type of medication that reduces inflammation in the body, should also be cared for in a more intensive setting, such as an intermediate step-down care unit or intensive care unit (ICU).

In addition, beneficial practices may include the use of laxatives, taking plenty of rest, and temporarily avoiding sexual activity.

During the treatment of RCVS, certain practices should be avoided. These include giving patients drugs for migraines that constrict blood vessels (such as triptans or ergots) and initiating treatment with glucocorticoids for PACNS, a rare type of brain inflammation. Other avoidable measures include proceeding to brain biopsy, a test where a small amount of brain tissue is removed for testing, or using intra-arterial vasodilators, medications that widen blood vessels and improve blood flow. Using intra-arterial vasodilators can lead to reperfusion injury, which is damage caused when blood supply returns to tissue after a period of lack of oxygen. Thus, their use is best avoided.

When brain imagery reveals an unusual condition within the brain arteries, doctors must first determine if the cause is due to a variety of possible conditions like moyamoya disease, arteriosclerosis in the brain, inflammation due to an infection, or a condition called PACNS.

PACNS and another condition, RCVS, often appear similar. One way to differentiate between the two conditions is by the patient populations affected and their symptoms. For instance, RCVS is usually found in younger, predominantly female patients. This condition often presents after recognizable triggers such as certain medications or stress. People with RCVS usually complain about severe headaches that come in recurring thunderclap episodes. On the other hand, PACNS doesn’t commonly relate to any identified trigger, and less than 10% of patients with PACNS report this type of intense headaches.

Furthermore, detailed brain imagery is essential to differentiate between RCVS and PACNS. In comparison, about 70% of RCVS patients show abnormalities in their initial CT or MRI images, but all PACNS patients have unusual findings on their initial scans. PACNS patients more frequently have brain infarcts, while swelling within the brain or minor bleeding at the convexity are specific to RCVS. Another unique feature is the brighter-than-normal appearance of brain arteries on a particular type of MRI scan, observed in around 61% of RCVS patients but only 7% of PACNS patients.

What to expect with Reversible Cerebral Vasoconstriction Syndromes

Patients with a condition called RCVS (Reversible cerebral vasoconstriction syndrome) that was caused by trauma or surgery, or those displaying certain brain function deficits, are more likely to have long-lasting neurological issues. Because of this, it’s crucial that these patients are closely monitored.

Some people with RCVS experience ongoing mild headaches. The condition can lead to brain or subarachnoid (bleeding between the brain and surrounding tissue) hemorrhages, which are most likely to occur in the early stages of the condition. Specifically, brain bleeds typically occur within the first 3 days after headache starts, while subarachnoid bleeding usually happens within the first 10 days. If an individual with RCVS is going to experience an ischemic stroke (blocked blood flow to the brain), it generally happens about two weeks after their headaches start.

However, many individuals with RCVS completely recover without any lingering symptoms, as the abnormal blood vessel narrowing that characterizes the condition often goes away on its own. A patient’s symptoms and physical signs of the condition may not disappear at the same time; one could persist even after the other has resolved. By definition, symptoms and physical signs of RCVS don’t usually last longer than 3 months and typically go away in a matter of weeks.

Despite the generally positive long-term outlook and the tendency for the condition to resolve on its own, sometimes, patients can experience severe narrowing of their blood vessels. This can lead to ischemic stroke and, in rare cases, death.

Possible Complications When Diagnosed with Reversible Cerebral Vasoconstriction Syndromes

The considerable side effects typically happen within the initial week after the start of a headache in Reversible Cerebral Vasoconstriction Syndrome (RCVS). These are:

Localized swelling in the brain due to a non-aneurysmal subarachnoid hemorrhage (22%)
Disease affecting the white matter of the brain, called leukoencephalopathy (9%)
Bleeding within the brain, also known as intracerebral hemorrhage (6%)
Seizures (3%)
Temporary loss of blood flow to the brain, causing stroke-like syndrome or ischemic stroke (20%). Most of these ischemic incidents occur in the second week.

These complications are potentially dangerous; they may cause lasting neurological damage.

Preventing Reversible Cerebral Vasoconstriction Syndromes

Firstly, when a diagnosis of RCVS (Reversible cerebral vasoconstriction syndrome, a disease that causes a temporary series of severe headaches) is made, it’s important for patients to be educated about the condition. Patients should understand that this illness is usually short-lived and reversible in nature. They should also be made aware of potential triggers or causes, so they can try to avoid them or quickly tell their healthcare team if they come across them.

The patients should also learn about the medications used to manage the symptoms of RCVS. Calcium channel blockers, a type of medication used to relieve symptoms, are often prescribed. However, patients should avoid glucocorticoids (a type of steroid), as these could potentially worsen the condition.

Patients should also be advised to stay away from activities that might make their headaches worse. The education provided to each patient should be customized according to their personal needs, their understanding of health-related matters, and their preferred way of learning. The information provided should be clear and to the point. Patients should be encouraged to ask questions and seek clarification about anything they don’t understand. If feasible, it is helpful to involve family members or caregivers in the learning process to ensure that everyone involved in the patient’s care understands the condition and its management.

Frequently asked questions

The prognosis for Reversible Cerebral Vasoconstriction Syndrome (RCVS) is generally positive, as the condition often resolves on its own. Many individuals with RCVS completely recover without any lingering symptoms. However, in some cases, severe narrowing of blood vessels can lead to ischemic stroke and, rarely, death. Symptoms and physical signs of RCVS typically go away within a matter of weeks and do not usually last longer than 3 months.

Many different triggers can lead to Reversible Cerebral Vasoconstriction Syndrome (RCVS), including severe headaches, physical exertion, pregnancy, high blood pressure conditions, certain medications and substances, tumors, physical trauma, surgery, and certain medical conditions.

Signs and symptoms of Reversible Cerebral Vasoconstriction Syndrome (RCVS) include: - Sudden, severe headache, often referred to as a thunderclap headache (TCH) - Headaches that reach their worst within a minute and tend to repeat, often weekly, for up to a month - Decrease in intensity and frequency of headaches over time - Seizures or neurological issues within the first 10 days of recurring headaches - Difficulty with speech or weak muscles on one side of the body, indicating possible brain injury - Visual symptoms such as blurry vision, temporary blindness, and blind spots - Signs of broad neurological trouble, including seizures and encephalopathy (brain disease that alters brain function) - Seizures in RCVS are characterized by muscle stiffening and violent shaking but are usually self-limiting - Seizures are less common in RCVS compared to another similar condition called PRES (Posterior reversible encephalopathy syndrome) - Possible triggers for RCVS include recent surgery (especially brain surgery), childbirth, medications, and recreational substances - Certain medications and substances, both legal and illegal, have been linked with RCVS - Family medical history is important to consider, as RCVS can be associated with inheritable conditions like pheochromocytoma and porphyria Additionally, a 2019 study introduced a rating scale called the RCVS2 score, which assesses the likelihood of RCVS diagnosis based on five factors: the presence of TCH, involvement of arteries inside the skull, being female, identifying a trigger that constricts blood vessels, and the presence of bleeding around the brain. A score of 5 or higher on the RCVS2 score indicates a 90% chance of RCVS diagnosis.

The types of tests that are needed for Reversible Cerebral Vasoconstriction Syndrome (RCVS) include: - RCVS2 score to assess the likelihood of RCVS in recurrent thunderclap headaches - Lumbar puncture to collect and analyze the fluid surrounding the brain and spinal cord - Brain biopsy to remove a small sample of brain tissue (invasive diagnostic test) - Urgent brain and blood vessel imaging to rule out life-threatening conditions - Angiography to visualize affected arteries and look for the characteristic "sausage on a string" appearance - Transcranial ultrasound to reveal elevated blood flow It is important to note that the preferred approach is to avoid unnecessary invasive tests and focus on treating the symptoms and removing triggers of the condition. Long-term medication for seizures is not usually necessary unless there is irreversible brain injury.

A doctor needs to rule out the following conditions when diagnosing Reversible Cerebral Vasoconstriction Syndrome (RCVS): - Ischemic stroke - Subarachnoid or intracerebral hemorrhage - Posterior reversible encephalopathy syndrome - Primary angiitis of the central nervous system (PACNS) - Aneurysmal subarachnoid hemorrhage - Pituitary apoplexy - Cervical artery dissection - Meningitis - Cerebral venous sinus thrombosis

The side effects when treating Reversible Cerebral Vasoconstriction Syndrome (RCVS) include: - Localized swelling in the brain due to a non-aneurysmal subarachnoid hemorrhage (22%) - Disease affecting the white matter of the brain, called leukoencephalopathy (9%) - Bleeding within the brain, also known as intracerebral hemorrhage (6%) - Seizures (3%) - Temporary loss of blood flow to the brain, causing stroke-like syndrome or ischemic stroke (20%). Most of these ischemic incidents occur in the second week. These complications can be potentially dangerous and may cause lasting neurological damage.

You should see a neurologist for Reversible Cerebral Vasoconstriction Syndrome.

The occurrence of Reversible Cerebral Vasoconstriction Syndrome (RCVS) could range between 7% to 54%, but it varies significantly.

The treatment for Reversible Cerebral Vasoconstriction Syndrome (RCVS) is largely focused on recognizing it as a condition that usually gets better on its own. The preferred approach is to treat the symptoms rather than introducing general treatments. It is important to remove any identified triggers of the condition and to treat headaches and high blood sugar levels, as both can potentially worsen patient outcomes. Long-term medication for seizures is not usually necessary unless there is irreversible brain injury. Patients undergoing glucocorticoid therapy should be cared for in a more intensive setting. Beneficial practices may include the use of laxatives, rest, and temporarily avoiding sexual activity. Certain practices should be avoided, such as giving patients drugs that constrict blood vessels, initiating treatment with glucocorticoids for a rare type of brain inflammation, proceeding to brain biopsy, or using intra-arterial vasodilators.

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a condition that causes severe and abrupt headaches that last for days to weeks. It occurs due to tightening of the blood vessels in the brain. RCVS is usually temporary and goes away on its own.

Cerebrovascular Dysregulation encephalopathy Health Seizure Severe Headache