Neurology and Neuro Surgery

Biography

Biography: Sylvain Lanthier

Abstract

Primary angiitis of the CNS (PACNS) is an idiopathic, single-organ vasculitis of small and medium-sized vessels. Diagnosis of PACNS is a real challenge because it is rarer than several other potential mimicking conditions. Clinical presentation are variable, including persistent headaches, cognitive decline, multifocal deficits and seizures. Brain lesions on MRI and inflammation of the cerebrospinal fluid are common findings, whereas angiographic changes may be documented mainly when medium-sized arteries are affected. None of these manifestations are specific for PACNS. CNS biopsy can prove the diagnosis when small arteries are involved, but this procedure is invasive and associated with potential false-negative results due to skipped lesions. Complementarily, gadolinium-enhancement of medium-sized arteries can be documented by high-resolution MRI. Treatment of PACNS is also challenging, aiming at minimizing the risks of treatment failure and recurrent vasculitis due to insufficient immunosuppression while avoiding adverse effects of potent immunosuppressant agents. Other single-organ vasculitides of the CNS comprise the amyloid-beta-related angiitis (ABRA) and the inflammatory form of cerebral amyloid angiopathy (iCAA). The nosological limits of PACNS, ABRA and iCAA remain misunderstood. The INTERnational Study on Primary Angiitis of the CEntral nervous system (INTERSPACE) is an international, multi-center, prospective, currently ongoing, cohort study. INTERSPACE was designed to describe clinical manifestations, investigation results, diagnostic processes, misdiagnoses, treatments and outcomes of clinically-suspected or biopsy-proven PACNS. Its primary objective is to identify predictors of death or dependence after 1 year of clinical follow-up. To join INTERSPACE, study investigators are invited to contact the Coordination Centre: sylanthier@gmail.com.