Cerebrovascular Manifestations of Neurosarcoidosis – An Overview

Neurosarcoidosis (NS) is defined as involvement of either central or peripheral nervous system by non-caseating granulomatous inflammation. They are seen clinically in 5-10% of those with sarcoidosis, though sub-clinical involvement is commoner. Cerebrovascular manifestations of neurosarcoidosis (CVNS) are considered even rarer but availability of novel imaging techniques and awareness of potential imaging markers for NS (yet to be validated), has resulted in better recognition. CVNS can occasionally be the presenting manifestation of sarcoidosis. Despite being rare, those with CVNS may have higher morbidity and mortality compared to other forms of NS. In this review, we aim to describe the clinical features, approach to diagnosis, mimics, investigation and management of CVNS, while attempting to identify features that differentiate central nervous system vasculitis associated with NS (CNSV-NS) from primary central nervous system vasculitis (PCNSV), a close diagnostic mimic.