Introduction
Reversible cerebral vasoconstriction syndrome (RCVS) combines a group of conditions that show reversible multifocal narrowing of the cerebral arteries with clinical manifestations that typically include thunderclap headache and sometimes include neurologic deficits related to brain edema, stroke, or seizure. The clinical features are usually benign at the start of the disease, although major strokes can result in severe disability or death in a minority.
What Is Reversible Cerebral Vasoconstriction Syndrome?
Reversible cerebral vasoconstriction syndrome (RCVS) is a rare chronic disease that occurs as the result of a constriction (tightening) of the vessels that supply blood to the brain. The main symptom of reversible cerebral vasoconstriction syndrome is sudden, severe, and disabling headaches which are sometimes called thunderclap headaches. Strokes or bleeding into the brain may or may not be present. Reversible cerebral vasoconstriction syndrome can be reversed. Outcomes for this syndrome in the affected patients range from full recovery to permanent brain damage in other patients.
What Are the Causes of Reversible Cerebral Vasoconstriction Syndrome?
A disturbance in the control of the smooth muscle tone inside the brain’s blood vessel walls is thought to cause reversible cerebral vasoconstriction syndrome. There is no evidence of inflammation or changes within the structure of the brain’s blood vessels or tissue among persons with reversible cerebral vasoconstriction syndrome. 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 reversible cerebral vasoconstriction syndrome may include the use of prescription, over-the-counter, or illegal drugs that can cause constriction of the arteries. Reversible cerebral vasoconstriction syndrome also may be linked to internal factors such as tumors, which secrete substances that, in turn, constrict blood vessels.
What Are the Signs and Symptoms of Reversible Cerebral Vasoconstriction Syndrome?
The followings are the signs and symptoms of reversible cerebral vasoconstriction syndrome.
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Headache.
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Strokes.
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Transient ischemic attacks.
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Weakness.
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Problems with eyesight.
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Seizures.
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Focal neurological deficits.
What Is the Pathophysiology of Reversible Cerebral Vasoconstriction Syndrome?
The pathophysiology of the onset of headache and the prolonged but reversible vasoconstriction is not still known. Reversible angiographic narrowing investigation shows an abnormality in the control of cerebrovascular tone. It remains unclear whether the angiographic abnormalities trigger or result from severe headaches, but there is certainly a close relationship. The anatomic basis for explaining vasoconstriction and headaches is the innervation of cerebral blood vessels with sensory afferents from the trigeminal nerve (V1) and dorsal root of C2. Cerebral vasoconstriction, when severe or progressive, may result in ischemic stroke and, in some cases, brain hemorrhages that probably reflect postischemic reperfusion injury due to the dynamic and reversible nature of the arterial narrowing. Some patients develop convex subarachnoid hemorrhages, presumably from the rupture of small surface arteries undergoing dynamic vasoconstriction-vasodilation.
The pathophysiologies of reversible cerebral vasoconstriction syndrome and reversible posterior leukoencephalopathy syndrome (RPLS) may overlap as both entities can present with reversible brain lesions, including transient brain edema in patients with reversible cerebral vasoconstriction syndrome and transient cerebral angiographic abnormalities in patients with reversible posterior leukoencephalopathy syndrome.
What Are the Other Terms or Diseases Associated With Reversible Cerebral Vasoconstriction Syndrome?
Central nervous system (CNS) vasculitis can be very similar to reversible cerebral vasoconstriction syndrome. Central nervous system vasculitis is a problem that results in inflammation of walls of the blood vessel walls in the brain or spine. (the central nervous system is composed of the brain and spine). Central nervous system vasculitis often occurs in the following situations:
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It appears with other autoimmune diseases, such as systemic lupus erythematosus, dermatomyositis, and rheumatoid arthritis.
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Central nervous system vasculitis may come along with a viral or bacterial infection.
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It can be associated with systemic (affecting the whole body) vasculitic disorders like granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and Behcet’s syndrome.
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It may appear on its own; in such cases, it is referred to as primary angiitis of the central nervous system.
The following are the terms in which reversible cerebral vasoconstriction syndrome is reported in many cases.
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Migrainous vasospasm or migraine angiitis.
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Call-Fleming syndrome (or Call syndrome).
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Thunderclap headache-associated vasospasm.
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Drug-induced cerebral arteritis.
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Postpartum cerebral angiopathy.
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Benign angiopathy of the central nervous system.
What Are the Drugs Associated With Reversible Cerebral Vasoconstriction Syndrome?
The following are the prescribed drugs associated with reversible cerebral vasoconstriction syndrome.
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Antidepressants.
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Medications to treat migraines like Triptans, Isometheptene, and Ergotamines.
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Immunosuppressants such as Cyclophosphamide and Tacrolimus.
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Drugs to prevent bleeding after childbirth, like contraceptives.
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Anti-Parkinson’s medications like Bromocriptine and Lisuride.
Following are some common over-the-counter drugs and supplements that can cause constriction of cerebral arteries.
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Nasal decongestants (Pseudoephedrine, Ephedrine, and Phenylpropanolamine).
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Nicotine patches.
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Caffeine-containing energy drinks.
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Ginseng.
Illegal drugs associated with reversible cerebral vasoconstriction syndrome.
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Marijuana.
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Cocaine.
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Ecstasy.
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Amphetamine derivatives.
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Lysergic acid diethylamide (LSD).
What Are the Investigations Done to Diagnose Reversible Cerebral Vasoconstriction Syndrome?
The diagnosis of reversible cerebral vasoconstriction syndrome is made by performing the following investigations.
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Recording patient’s medical and personal history.
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A physical examination is done, and all the vitals are checked.
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A complete blood count test is performed to detect the infection and abnormality in the count of blood cells.
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Imaging tests including computed tomography (CT) scan head and magnetic resonance imagining (MRI) is performed. These imaging tests help to detect the developed parenchymal lesions, widespread cerebral vasoconstriction, nonaneurysmal subarachnoid hemorrhage, brain edema, and parenchymal hemorrhage.
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Other imaging modalities can also be done for more confirmation, like neuroimaging and cerebral angiography.
What Are the Treatment Modalities for Reversible Cerebral Vasoconstriction Syndrome?
According to recent studies, there is no cure for reversible cerebral vasoconstriction syndrome. Some supportive treatments can help to manage the symptoms, like controlling pain, seizures, blood pressure, headache, and others. Patient counseling can also help the patient to avoid the use of triggering factors like some medicated or illegal drugs.
Conclusion
Reversible cerebral vasoconstriction syndrome is a rare chronic group of disorders. It is mainly associated with vasoconstriction of the arteries in the brain and brain damage. There is no proven cure for this disorder, but supportive treatment can help. Always consult a doctor if a person starts developing some related signs and symptoms.
