Cerebellar leptomeningeal enhancement: An imaging finding of rapidly progressive Purkinje cell cytoplasmic autoantibody type 1 paraneoplastic cerebellar syndrome

J Neuroimmunol. 2024 Feb 15:387:578293. doi: 10.1016/j.jneuroim.2024.578293. Epub 2024 Jan 19.

Abstract

Purkinje cell cytoplasmic autoantibody type 1 (PCA1), also known as anti-Yo, is a 'high-risk' paraneoplastic antibody, associated with rapidly progressive cerebellar syndrome. In patients with this syndrome, various MRI abnormalities have been documented, including atrophy in the cerebellum and brainstem, T2 hyperintensity in the brainstem and spinal cord, and cranial nerve enhancement. This report introduces an imaging finding, cerebellar leptomeningeal enhancement, which was observed in all three cases at early stages. Despite neurological deterioration, all patients underwent immunotherapy, and subsequent follow-up MRI revealed resolution of the leptomeningeal enhancement, suggesting that this feature is distinct from meningeal carcinomatosis.

Keywords: Anti-Yo; Leptomeningeal enhancement; Paraneoplastic; Purkinje cell cytoplasmic autoantibody type 1; Rapidly progressive cerebellar syndrome.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies
  • Cerebellar Diseases* / diagnostic imaging
  • Cerebellar Diseases* / metabolism
  • Cerebellum / metabolism
  • Humans
  • Nerve Tissue Proteins
  • Paraneoplastic Cerebellar Degeneration* / diagnostic imaging
  • Paraneoplastic Cerebellar Degeneration* / metabolism
  • Paraneoplastic Syndromes*
  • Purkinje Cells / metabolism

Substances

  • Autoantibodies
  • Nerve Tissue Proteins