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

Adam J Stark; Bret C Mobley; James E Eaton

doi:10.1007/s00415-024-12283-7

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

J Neurol. 2024 Mar 13. doi: 10.1007/s00415-024-12283-7. Online ahead of print.

Authors

Adam J Stark¹, Bret C Mobley², James E Eaton³

Affiliations

¹ School of Medicine, Vanderbilt University, Nashville, TN, 37232, USA.
² Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, TN, 37232, USA.
³ Department of Neurology, Vanderbilt University Medical Center, 1500 21St Ave South, Suite 2001, Nashville, TN, 37212, USA. james.e.eaton@vumc.org.

PMID: 38478031
DOI: 10.1007/s00415-024-12283-7

Abstract

Background and objective: 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.

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

Discussion: 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.

Keywords: Leukoencephalopathy; MRI; PACNS.

Publication types

Letter