Reversible Cerebral Vasoconstriction Syndrome (RCVS)
Definition and Clinical Presentation
RCVS is a rare neurological disorder characterized by recurrent severe thunderclap headaches and diffuse segmental narrowing of cerebral arteries that reverses within 3 months. 1 The condition affects predominantly middle-aged adults with female preponderance, particularly in the postpartum period. 2
Key Clinical Features:
- Recurrent thunderclap headaches are the hallmark presentation, though RCVS can manifest as single severe episodes, mild progressive headaches, or even without headache in severe cases presenting with seizures, focal deficits, confusion, or coma. 3
- Postural headaches occur in 83% of cases. 4
- Associated symptoms include nausea/vomiting (28%), seizures, and focal neurological deficits. 1, 5
Common Triggers:
- Vasoactive substances: serotonergic agents, sympathomimetic drugs, SSRIs, triptans, decongestants, marijuana, and cocaine. 6, 7, 2
- Postpartum state, exertion, sexual activity, coughing, showering. 5
- Blood transfusions in patients with chronic anemia. 8
Diagnosis
Diagnosis relies on demonstrating reversible multifocal intracranial arterial stenosis while excluding other causes of thunderclap headache. 3
Imaging Approach:
- Cerebral catheter digital subtraction angiography (DSA) is the gold standard, though CT angiography or MR angiography are reliable non-invasive alternatives. 5
- Brain imaging may show hemorrhagic or ischemic lesions in one-third to half of cases. 1
- Posterior reversible encephalopathy syndrome (PRES) frequently coexists with RCVS and shares common pathophysiology. 1
Critical Differential Diagnoses:
- Aneurysmal subarachnoid hemorrhage
- Cervical artery dissection
- Primary angiitis of the central nervous system (PACNS)
- Cerebral venous thrombosis 5
Treatment
The cornerstone of RCVS management is supportive care with bed rest, analgesics, and immediate removal of precipitating factors. 1 Glucocorticoids are an independent predictor of worse outcome and should be avoided. 1
Pharmacological Management:
- Calcium channel blockers (nimodipine or verapamil) reduce headache intensity but do not affect the time course of cerebral vasoconstriction. 1
- Blood pressure control is essential, particularly in patients with vasogenic edema. 8
- For persistent hypotension, intravenous phenylephrine (1-10 mcg/kg/min) or dopamine (5-15 mcg/kg/min) may be considered. 7
Invasive Interventions:
- Neurointerventional techniques should be reserved only for severe deteriorating cases. 1
- Avoid invasive procedures (central venous catheterization, lumbar puncture, bronchoscopy) during active coagulopathy if present. 7
Monitoring Requirements:
- Obtain complete blood count and inflammatory markers (ESR, CRP) to differentiate from other causes. 7
- Monitor electrolytes carefully if using medications affecting electrolyte balance. 7
- Close neurological monitoring is essential to detect early complications. 4
Complications and Prognosis
RCVS is usually benign and self-limited, but 5-10% of patients develop permanent neurological deficits and rare deaths occur. 1
Major Complications:
- Focal subarachnoid hemorrhage
- Intracerebral hemorrhage
- Ischemic stroke
- Intraparenchymal hemorrhage (22% of cases)
- Seizures (22% of cases)
- Subdural hematomas (11% of cases) 4
Mortality:
The mortality rate of RCVS is approximately 3%, typically associated with life-threatening complications such as cerebral venous thrombosis. 4 Most patients have favorable outcomes with complete resolution of symptoms and imaging findings within 3 months. 1, 2
Important Caveats:
- Spontaneous intracranial hypotension can develop, manifesting as postural headaches and potentially leading to cerebral venous thrombosis. 4
- Persistent CSF leaks may require epidural blood patch (effective in 42% of cases) or open surgical repair. 4
- The absence of thunderclap headache should not exclude RCVS diagnosis—suspect RCVS in any patient with unusual headache patterns or cryptogenic stroke with convexity subarachnoid hemorrhage. 3