How to manage Reversible Cerebral Vasoconstriction Syndrome (RCVS) with leukocytosis?

Management of Reversible Cerebral Vasoconstriction Syndrome (RCVS) with Leukocytosis

The management of RCVS with leukocytosis should focus on removing precipitating factors, providing supportive care with calcium channel blockers, and avoiding glucocorticoids which can worsen outcomes.

Clinical Features and Diagnosis

• RCVS is characterized by recurrent severe thunderclap headaches with diffuse segmental narrowing of cerebral arteries that resolves within 3 months 1
• Common triggers include serotonergic agents, sympathomimetic drugs, marijuana, SSRIs, triptans, and decongestants 2, 1
• Leukocytosis in RCVS may be a manifestation of the inflammatory response or related to medications used to treat the condition 3
• Despite established diagnostic criteria suggesting normal CSF findings, RCVS can present with CSF leukocyte levels >10/mm³, which can lead to misdiagnosis as cerebral vasculitis 4

Initial Assessment and Workup

• Obtain complete blood count to evaluate leukocytosis 3
• Perform inflammatory markers testing (ESR, CRP) to help differentiate from other causes 3
• Conduct neuroimaging with MRI/MRA as studies of choice; catheter angiography should not be implemented routinely 5
• Consider transcranial color-coded sonography - patients with mean flow velocity of middle cerebral artery >120 cm/s have greater risk of ischemic complications 5
• Perform lumbar puncture if not contraindicated, but be aware that CSF pleocytosis can occur in RCVS and should not automatically lead to a diagnosis of vasculitis 4

Management of RCVS with Leukocytosis

First-line Treatment

• Immediately discontinue any potential triggering medications or substances 1, 6
• Provide calcium channel blockers as first-line therapy:
  • Nimodipine (oral or intravenous) has been reported to reduce headache intensity 1, 6
  • Verapamil is an alternative calcium channel blocker option 1
• Implement bed rest and provide appropriate analgesics for pain control 1

Management of Leukocytosis

• Monitor complete blood count regularly to track leukocytosis 3
• Investigate for other causes of leukocytosis including infections, stress response, or medication effects 3
• Avoid glucocorticoids for treating leukocytosis as they are an independent predictor of worse outcomes in RCVS 1

Management of Complications

• Monitor for potential complications which occur at different time courses:
  • Early complications (first week): cortical subarachnoid hemorrhage (22%), intracerebral hemorrhage (6%), seizures (3%), and reversible posterior leukoencephalopathy (9%) 6
  • Later complications (second week): ischemic events including TIAs (16%) and cerebral infarction (4%) 6
• For severe deteriorating cases, consider invasive neurointerventional techniques, but these should be reserved as last resort 1

Special Considerations

• Avoid invasive procedures such as central venous catheterization, lumbar puncture, and bronchoscopy during active coagulopathy if present 3
• Carefully monitor electrolytes and maintain normal ranges if using medications that may affect electrolyte balance 3
• Be aware that women may have different clinical presentations with higher rates of stroke and cortical subarachnoid hemorrhage 6
• Recognize that despite leukocytosis, RCVS is typically self-limited with favorable outcomes in most patients 1, 5

Follow-up and Monitoring

• Continue calcium channel blockers for 4-12 weeks 1, 6
• Perform follow-up neuroimaging to confirm resolution of vasoconstriction, which typically occurs within 1-3 months 1
• Monitor for recurrent thunderclap headaches, which typically resolve within 1-2 weeks 5
• Long-term follow-up is generally not necessary as relapse is rare, reported in only a small proportion of patients 5