RCVS

Case: 44 years old female is admitted to the ICU with severe, sudden headache in the occipital region, associated with nausea and photosensitivity, triggered after sexual activity and orgasm. MRI of the brain showed vasogenic edema and sulcal hyperintensities on fluid-attenuated inversion recovery (FLAIR) (dot sign). Patient gets diagnosed with reversible cerebral vasoconstriction syndrome (RCVS). As primary angiitis of the central nervous system (PACNS) is also under consideration, administering glucocorticoids should be considered.

A) True

B) False

Answer: B

Although PACNS shares various features with RCVS, administering glucocorticoids is not recommended. It is vital to perform a thorough differential diagnosis between the two conditions by using angiography and the RCVS-2 score. A classic clinical difference is that PACNS usually have an insidious progressive clinical course with chronic headaches and rarely have a thunderclap headache, which is typical of RCVS. A neurologist should be consulted for such a critical differential. 

Glucocorticoids are associated with worse outcomes in RCVS. Fortunately, a period of observation after a dramatic presentation provides time to differentiate between the two situations and does not significantly affect the outcome by delaying glucocorticoid administration. Empiric glucocorticoid therapy should be carried out only if there is a rapidly worsening clinical course while the diagnosis remains uncertain. 

Aneurysmal subarachnoid hemorrhage is another differential to be considered closely. Angiography is an essential tool..

#neurology

References:

1. Singhal AB. Reversible cerebral vasoconstriction syndrome: A review of pathogenesis, clinical presentation, and treatment. Int J Stroke 2023; 18:1151.

2. Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol 2011; 68:1005.

3. Singhal AB, Topcuoglu MA. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome. Neurology 2017; 88:228.

4. Ribas MZ, Paticcié GF, de Medeiros SDP, de Oliveira Veras A, Noleto FM, Dos Santos JCC. Reversible cerebral vasoconstriction syndrome: literature review. Egypt J Neurol Psychiatr Neurosurg. 2023;59(1):5. doi: 10.1186/s41983-023-00607-9. Epub 2023 Jan 11. PMID: 36647436; PMCID: PMC9833030.