Reversible cerebral vasoconstriction syndrome is a condition associated with severe headaches. The intensity of this pain is so severe that it is sometimes referred to as annihilation headache. Reversible cerebral vasoconstriction syndrome is also referred to in some cases by the abbreviation RCVS or the synonymous term Call-Fleming syndrome. As part of the disease, muscles in brain vessels contract, resulting in severe headaches. Other neurological symptoms do not occur in every case.
What is reversible cerebral vasoconstriction syndrome?
Reversible cerebral vasoconstriction syndrome is characterized by extremely severe headaches, known as annihilation headaches. These may be accompanied by other neurologic disorders. These include, for example, a stroke or seizure as well as a so-called subarachnoid hemorrhage. A typical feature of the disease, however, is that the symptoms usually disappear again within a period of about three months. The disease has already been observed in persons between the 10th and 80th decade of life. However, the typical complaints occur most frequently in the 40th year of life. It has been shown that more female than male patients are affected by the syndrome. Exact data on the frequency of reversible cerebral vasoconstriction syndrome are not yet available. However, the disease is not all that rare. For example, it has been found that the condition often occurs in women after childbirth or in the setting of eclampsia. The syndrome also occurs more frequently after the consumption of serotonergic and adrenergic agents or the use of amphetamines and cocaine.
Causes
The causes of reversible cerebral vasoconstriction syndrome have not been conclusively investigated. Basically, annihilation headaches form in the back of the head in most cases and extend from there across the head.
Symptoms, complaints, and signs
In the context of reversible cerebral vasoconstriction syndrome, various symptoms and complaints occur in affected patients. Headache is usually the central symptom. They often start as destructive headaches. These pains start suddenly and develop after a short time to extremely strong pains. The origin of these headaches is usually in the back of the head. However, the pain gradually spreads to the entire head. In connection with this, other symptoms are possible, for example, confusion, vomiting, nausea, a tendency to collapse, and sensitivity to noise and light. These headaches resolve after a few minutes or days. On average, the pain in reversible cerebral vasoconstriction syndrome lasts for several hours. During the first few weeks, there are usually several attacks of annihilation headache. In the intervening periods, patients usually suffer only mild headaches. In addition, other complaints are possible. These include, for example, deficits of a focal nature as well as epileptic seizures. Dangerous complications, such as strokes, also sometimes occur.
Diagnosis and course of the disease
Reversible cerebral vasoconstriction syndrome can be diagnosed using a variety of investigative measures and methods. If individuals notice typical symptoms in themselves, a physician should be consulted quickly. In the first step of diagnosis, the physician takes a medical history with the patient. In the course of this discussion, he asks about the characteristic complaints, lifestyle and consumption of stimulants, past illnesses and genetic dispositions. In this way, the specialist obtains significant information regarding the disease at hand. The next step is to focus on the clinical complaints of the affected patient. If the person describes typical symptoms of reversible cerebral vasoconstriction syndrome, a tentative diagnosis is already possible. To confirm this suspicion, various examinations are used. For example, the blood is examined for inflammatory markers and specific antibodies. The urine is also analyzed. Finally, imaging procedures of the brain are used.In the course of these examinations, diffuse vasoconstrictions are revealed in many cases. Magnetic resonance imaging and computer tomography are also usually performed. In some cases, infarcts caused by bleeding are visible here. Edema in the brain is also possible. With regard to the differential diagnosis, subarachnoid hemorrhage, torn neck vessel walls, and primary angiitis of the central nervous system should be excluded in the first instance.
Complications
Reversible cerebral vasoconstriction syndrome is a very serious condition, but it usually resolves on its own after approximately three months. However, the spasmodic constrictions of the cerebral vasculature can lead to strokes. These are the most important complications in reversible cerebral vasoconstriction syndrome. Both hemorrhagic and ischemic strokes occur. While a hemorrhagic stroke is characterized by cerebral hemorrhage, in an ischemic stroke the blood supply is interrupted by vasoconstriction. In both cases, however, brain tissue dies, which can have a long-term negative impact on physical and mental development. Approximately five percent of patients even suffer from life-threatening forms of the disease with multiple strokes and brain edema. In addition, single epileptic seizures are also possible in rare cases. Although the main symptom of the disease is a sudden-onset annihilation headache, no permanent physical damage usually remains if the disease progresses without complications. However, the headache is so severe that it greatly affects the quality of life during the time of the attack. The accompanying symptoms, such as nausea, vomiting, tendency to collapse or states of confusion, also lead to a drastic reduction in physical and mental performance. This can cause severe psychological problems in some patients. In this case, there is a risk that depression and suicidal thoughts may also develop on this basis.
When should you go to the doctor?
Reversible cerebral vasoconstriction syndrome usually results in such a severe headache that it is rare for sufferers to refrain from seeing a doctor. It is also not advisable to refrain from visiting the doctor after a so-called annihilation headache. The trigger is a muscle contraction in the head. But the consequences of a reversible cerebral vasoconstriction syndrome can be so severe that a visit to the doctor should be made immediately. This is especially true if there are concomitant other neurologic abnormalities. These may include confusion, visual loss, vomiting, epileptic seizures, cerebral hemorrhage, or stroke. Reversible cerebral vasoconstriction syndrome usually resolves within three months. But the suffering during this period can be considerable. It depends on the duration and intensity of the headache attacks. The possible causes of this phenomenon must be determined, if possible, by the medical history. If none are found to explain the condition, this is not unusual. Reversible cerebral vasoconstriction syndrome has not been adequately studied. Symptomatic medical treatment may need to be initiated. This is because reversible cerebral vasoconstriction syndrome is a serious neurological condition that can lead to strokes. It is caused by the spasmodic constrictions with which muscles in blood vessels in the brain contract.
Treatment and therapy
Therapy for reversible cerebral vasoconstriction syndrome is usually medicinal. It should be noted that no guidelines have yet been established for the treatment of the disease. In any case, early diagnosis and adequate therapy are important. This is primarily a symptomatic treatment. A central goal of therapy for reversible cerebral vasoconstriction syndrome is also to minimize factors that promote vasoconstriction. This includes, for example, avoiding stress, sports, and other physical exertion. In addition, so-called vasoactive drugs are used. Analgesics and benzodiazepines are also often used. If patients suffer from epileptic seizures, antiepileptic drugs are also administered.The prognosis of reversible cerebral vasoconstriction syndrome is relatively good, as it usually resolves within a few days, weeks, or months.
Prevention
Reversible cerebral vasoconstriction syndrome is almost impossible to prevent. This makes it all the more important to consult a physician if symptoms develop, so that appropriate therapy for the symptoms can be started.
Follow-up
In the follow-up of reversible cerebral vasoconstriction syndrome, it is important to take the medications prescribed by a physician exactly as recommended. While there are no direct preventive measures, close monitoring of symptoms helps to detect relapse early. Patients should see a doctor in the short term if they experience any irregularities. In addition, they can limit the factors that threaten their health by reducing stress and physical exertion. If epileptic seizures occur, patients must take the prescribed antiepileptic drugs. There is also a possibility of using alternative healing methods, but it is necessary to have a detailed consultation with the doctor. It is very helpful to integrate such therapy into everyday life, whether it is yoga or other relaxation exercises. The gentle body movement and muscle strengthening lead to hormone release, which reduces pain sensitivity. Many doctors also recommend these exercises for their psychotherapeutic benefits. The relaxing techniques also make patients feel better mentally. That’s why it’s important for sufferers to do their exercises regularly. This improves their condition in the long term. By attending a therapy group and involving relatives and friends, patients feel even better cared for, which has a beneficial effect on their health.
Here’s what you can do yourself
Although reversible cerebral vasoconstriction syndrome usually resolves within a few months, affected patients suffer a great deal of pain. In addition to medical treatments, alternative therapeutic methods can be used. These are taught by specially trained experts such as doctors and therapists and can then be incorporated into individual daily routines independently. Examples may include performing yoga exercises and other physical therapies. The movement and strengthening of the body releases hormones that suppress the sensation of pain. Another positive aspect lies in the psychological significance of such exercises. Mental relaxation, for example through meditation, can help prevent depression. It is important to carry out these measures regularly. This is the only way to achieve long-term effects. But the social environment of affected persons should not be ignored in a holistic treatment. Since patients often withdraw as a result of the disease, it is important to counteract this. Participation in therapy groups and contact with friends and relatives is particularly important during this time. In this way, new courage to face life can be found despite the disease.
