Reversible Cerebral Vasoconstriction Syndrome: A Retrospective study from South India

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare cause of stroke characterized by headache, seizures, focal deficits, or encephalopathy. Very little is known about this rare condition from the Indian subcontinent. Here, we present the clinical and imaging characteristics and short-term outcomes of RCVS patients from South India.

A single-center retrospective study of all consecutive subjects with a clinical-radiological diagnosis of RCVS from January 2014 to December 2023 with a 3-month completed follow-up was conducted. The clinical features, vascular imaging patterns, and outcomes of patients with ischemic and hemorrhagic forms of RCVS were compared.

Of the 22 patients who fulfilled Calabrese et al.'s criteria for RCVS, the majority were women with a mean age of 47.59 (±13.55) years. While headache was the most common presenting symptom in our cohort (18/22, 81.81%), 14/22 (63.6%) developed focal neurologic deficits during the course of illness. Four of 22 patients (18%) did not report headaches during the course of illness. The most common imaging finding at presentation was cortical subarachnoid hemorrhage (SAH) in 9/22 (40.9%), followed by infarcts in 6/22, (27.2%), while 12/22 (54.5%) patients developed new ischemic lesions on repeat imaging. Ischemic and hemorrhagic presentations of RCVS did not differ in terms of clinical presentation or outcome. All patients with ischemic lesions showed diffuse vasospasm on imaging, while those with SAH had both diffuse and focal vascular abnormalities.

We present the largest single series of RCVS from India, with a favorable short-term outcome. Although the most common vascular abnormality is diffuse vasospasm, it can remain focal in a quarter of patients.