Reversible cerebral vasoconstriction syndrome

Overview

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a neurological disorder characterized by sudden, severe "thunderclap" headaches and reversible narrowing (vasoconstriction) of the cerebral arteries. The condition typically affects middle-aged adults and is more common in women. Although most patients recover completely, RCVS can sometimes lead to serious complications such as stroke, brain hemorrhage, or seizures.

RCVS is often self-limiting, with symptoms and vascular abnormalities resolving within 1 to 3 months. It is frequently triggered by certain medications, illicit drugs, or postpartum hormonal changes. Prompt recognition and management are essential to reduce the risk of complications and improve outcomes.

Causes

The exact cause of RCVS is not fully understood, but it is believed to involve a temporary dysregulation of cerebral vascular tone, resulting in segmental vasoconstriction of the brain arteries. Several factors and triggers have been associated with the condition:

Common Triggers:

• Vasoactive substances: Selective serotonin reuptake inhibitors (SSRIs), nasal decongestants, triptans (migraine medications), and energy drinks

• Illicit drug use: Cocaine, cannabis, amphetamines

• Postpartum state: Especially within the first 6 weeks after delivery

• Blood pressure fluctuations: Sudden changes in blood pressure, including hypertensive crises

• Physical exertion: Intense exercise, sexual activity, or emotional stress

Other Associated Conditions:

• Pre-eclampsia and eclampsia

• Use of immunosuppressive or chemotherapy agents

• Trauma or neurosurgical procedures

Symptoms

The hallmark symptom of RCVS is a sudden, explosive headache known as a "thunderclap headache," which reaches peak intensity within seconds to a minute. These headaches may occur repeatedly over days to weeks.

Primary Symptoms:

• Thunderclap headaches: Severe, sudden headaches that may start at the back of the head and spread

• Recurrent headaches: Multiple episodes over 1–3 weeks

Other Possible Symptoms:

• Nausea and vomiting

• Visual disturbances: Blurred vision, photophobia

• Seizures

• Transient neurological deficits: Weakness, speech difficulties, numbness

• Altered consciousness or confusion

Complications (in severe cases):

• Ischemic stroke

• Intracerebral hemorrhage

• Subarachnoid hemorrhage (SAH)

• Posterior reversible encephalopathy syndrome (PRES)

Diagnosis

Diagnosing RCVS can be challenging, as its symptoms overlap with other serious neurological conditions such as subarachnoid hemorrhage or vasculitis. A combination of clinical presentation, imaging studies, and follow-up assessments is typically required.

Initial Evaluation:

• CT scan of the head: To exclude hemorrhage or mass lesions

• Lumbar puncture: To rule out subarachnoid hemorrhage if CT is negative

Definitive Imaging:

• CT angiography (CTA) or MR angiography (MRA): Reveals segmental narrowing and dilatation ("string of beads" appearance) of cerebral arteries

• Conventional cerebral angiography: Most sensitive method for confirming vasoconstriction

Follow-Up:

• Repeat imaging after 1 to 3 months typically shows resolution of vasoconstriction, confirming the diagnosis of RCVS

Differential Diagnosis:

• Primary angiitis of the central nervous system (PACNS)

• Aneurysmal subarachnoid hemorrhage

• Migraine or cluster headaches

• Intracerebral hemorrhage or ischemic stroke

Treatment

The treatment of RCVS focuses on symptom control, removing any potential triggers, and managing complications. Most patients improve without the need for aggressive interventions.

1. Elimination of Triggers:

• Discontinue all vasoactive drugs (e.g., triptans, SSRIs, decongestants)

• Avoid physical exertion and stressors that may provoke headaches

2. Pain Management:

• Analgesics: NSAIDs or acetaminophen for headache relief

• Calcium channel blockers: Nimodipine or verapamil may help reduce vasospasm and alleviate headache intensity

3. Blood Pressure Management:

• Control of high blood pressure, especially in hypertensive crises or postpartum patients

4. Seizure and Stroke Management:

• Anticonvulsants for seizure control

• Standard stroke care if ischemic or hemorrhagic complications occur

5. Hospitalization:

• Advised for initial diagnosis, monitoring for complications, and initiation of treatment

Prognosis

The prognosis for RCVS is generally favorable. Most patients experience a complete resolution of symptoms and vascular abnormalities within 1 to 3 months. However, the risk of stroke or hemorrhage though relatively low necessitates early diagnosis and close monitoring during the acute phase.

With prompt treatment and avoidance of triggering substances, long-term complications are rare. Recurrence is uncommon but possible, especially if the triggering factors are reintroduced. Patients should receive counseling on lifestyle modifications and medication safety to reduce recurrence risk.

In cases complicated by stroke, permanent neurological deficits may occur, depending on the severity and location of brain injury. Overall, the majority of patients recover well with appropriate care.