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

J Neurol. 2024 Jun;271(6):3648-3652. doi: 10.1007/s00415-024-12283-7. Epub 2024 Mar 13.

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

  • Case Reports
  • Review

MeSH terms

  • Brain / diagnostic imaging
  • Brain / pathology
  • Humans
  • Leukoencephalopathies* / diagnostic imaging
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Vasculitis, Central Nervous System* / complications
  • Vasculitis, Central Nervous System* / diagnostic imaging

Supplementary concepts

  • Primary angiitis of the central nervous system