High-Resolution Vessel Wall Imaging in Primary CNS Vasculitis: A Case Series

Abstract

High-resolution vessel wall imaging (HRVWI) is a relatively new technique in which vessel walls can be imaged suppressing the cerebrospinal fluid and blood signals. If there is inflammation of the vessel wall (vasculitis) it will show contrast enhancement. This is the only modality which is available to study vessel walls. All angiography techniques including Magnetic Resonance Angiography(MRA), Contrast computerized tomography angiography (CT -Angiography), and digital subtraction angiography (DSA) shows only the state of the lumen of the blood vessel. Involvement of blood vessels in Central Nervous system (CNS) can occur in systemic vasculitis (like microscopic polyangiitis, Polyarteritis nodosa, etc) where there is evidence of other organ involvement or primary CNS vasculitis (PCNSV) in which only involvement of the brain and spinal cord occurs. PCNSV is a rare disease and till now only the brain biopsy is the gold standard for definitive diagnosis. Brain biopsy is an invasive procedure, not always available, and many patients don’t give consent for the procedure. HRWVI can complement the angiography in the diagnosis of CNS vasculitis, especially PCNSV. Here we describe a case series of 4 cases of CNS vasculitis (3 cases of possible PCNSV and one vasculitic moya- moya syndrome) who had presented with stroke, All had abnormal Cerebrospinal fluid (CSF) and HRVWI showed contrast enhancement of the vessel wall indicating the possibility of vasculitis. In two cases, there was involvement of medium-sized arteries (middle cerebral artery), one patient had unilateral internal carotid artery (ICA) involvement and one had bilateral supra- clinoid stenosis of ICA associated with hyperthyroidism (vasculitic Moyamoya syndrome).

Keywords

• primary cns vasculitis
• high resolution vessel wall imaging
• moya moya syndrome