Progressive leukoencephalopathy as a manifestation of primary angiitis of the central nervous system: case report and review of the literature

Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis solely affecting the vessels of the brain, spinal cord, and leptomeninges. A range of magnetic resonance imaging (MRI) features have been associated with PACNS, including cerebral infarction, hemorrhage, and parenchymal or leptomeningeal contrast enhancement.

We describe a 51-year-old man with a case of PACNS manifesting as akinetic mutism with progressive leukoencephalopathy.

Progressive leukoencephalopathy has not been well defined as a manifestation of PACNS. We review a small number of cases with comparable features, providing additional context on this PACNS manifestation with consideration of clinical subtypes.