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

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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.

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Authors and Affiliations

  1. School of Medicine, Vanderbilt University, Nashville, TN, 37232, USA

    Adam J. Stark

  2. Department of Pathology, Microbiology, and Immunology, Vanderbilt University Medical Center, Nashville, TN, 37232, USA

    Bret C. Mobley

  3. Department of Neurology, Vanderbilt University Medical Center, 1500 21St Ave South, Suite 2001, Nashville, TN, 37212, USA

    James E. Eaton

Authors
  1. Adam J. Stark
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  2. Bret C. Mobley
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  3. James E. Eaton
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Correspondence to James E. Eaton.

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The authors declare no competing financial interests.

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Stark, A.J., Mobley, B.C. & Eaton, J.E. Progressive leukoencephalopathy as a manifestation of primary angiitis of the central nervous system: case report and review of the literature. J Neurol 271, 3648–3652 (2024). https://doi.org/10.1007/s00415-024-12283-7

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  • DOI: https://doi.org/10.1007/s00415-024-12283-7

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