Cerebrospinal fluid findings in reversible cerebral vasoconstriction syndrome: a way to differentiate from cerebral vasculitis?

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by an acute onset of severe headache and multi-focal segmental vasoconstriction of cerebral arteries resolving within 12 weeks. Diagnostic criteria include normal or near-normal findings in cerebrospinal fluid (CSF) analysis, especially leucocyte levels < 10/mm³. Distinguishing RCVS from primary angiitis of the central nervous system (PACNS) is essential to avoid unnecessary and sometimes unfavourable immunosuppressive treatment. We reviewed retrospectively the clinical and diagnostic data of 10 RCVS patients who presented in our neurological department from 1 January 2013 to February 2017. The main purpose was to verify whether CSF leucocyte counts < 10/mm³ serve to discriminate RCVS from PACNS. Five of six patients who underwent lumbar puncture presented with CSF leucocyte levels ≥ 10/mm³. Two patients had a history of misinterpretation of CSF pleocytosis as cerebral vasculitis and of immunosuppressive treatment. A complete restitution of cerebral vasoconstriction was evident in all. No patient had further cerebral strokes or bleedings without immunosuppressive treatment over more than 12 weeks. Despite the established diagnostic criteria, RCVS can manifest with CSF leucocyte levels > 10/mm³. Careful anamnesis and the response of 'vasculitis-like angiography' to nimodipine given as a test during angiography and as oral medication are key to differentiate RCVS from cerebral vasculitis.

Review on the Diagnosis and Treatment of Reversible Cerebral Vasoconstriction Syndrome in Children and Adolescents

“…Conversely, there was a small case series that did identify several adults with RCVS and somewhat higher CSF leukocyte levels, ranging from 10 to 29/μL; therefore, using clinical context is crucial in the CSF interpretation. 36 Brain biopsy is not recommended unless the clinical suspicion for vasculitis is high. RCVS has normal arterial histology based on prior data collected from brain biopsy or autopsy.…”

Extended stereotactic brain biopsy in suspected primary central nervous system angiitis: good diagnostic accuracy and high safety

“…The good safety data on extended stereotactic brain biopsies in our cohort also might be influenced by the selection of the patients for biopsy, which means caution is required when comparing our safety data with data from other PACNS-studies. A relevant limitation of our study definitely is the quite low rate of conventional angiography of the cerebral arteries (52.2%) before brain biopsy, since some differential diagnoses of PACNS such as Divry van Bogaert syndrome or RCVS can only be diagnosed by conventional angiography [ 19 , 20 ]. Particularly for the differentiation of RCVS from PACNS conventional angiography with intraarterial nimodipin application is extremely helpful [ 20 ].…”

“…A relevant limitation of our study definitely is the quite low rate of conventional angiography of the cerebral arteries (52.2%) before brain biopsy, since some differential diagnoses of PACNS such as Divry van Bogaert syndrome or RCVS can only be diagnosed by conventional angiography [ 19 , 20 ]. Particularly for the differentiation of RCVS from PACNS conventional angiography with intraarterial nimodipin application is extremely helpful [ 20 ]. However, conventional angiography probably would not have been of additional diagnostic value in most of our cases, since most patients presented with small vessel disease.…”

Approach to Headache

“…CSF analysis in the majority of patients with vasculitis will be inflammatory, showing a modest lymphocytic pleocytosis, elevated protein level, and occasionally the presence of oligoclonal bands. 55 Though mild inflammation may also be seen in RCVS, 55 a more sterile sample would be an indicator RCVS is more likely than vasculitis, preventing the need for an invasive brain biopsy (the diagnostic gold standard of CNS vasculitis), and avoiding administration of immunomodulating treatment (as indicated for CNS vasculitis).…”