An autopsy case of progressive supranuclear palsy treated with monoclonal antibody against tau

Neuropathology. 2023 Aug;43(4):326-332. doi: 10.1111/neup.12890. Epub 2023 Jan 2.

Abstract

We report an autopsy case of progressive supranuclear palsy (PSP-Richardson syndrome). The individual had been enrolled in a phase 2 trial and received a monoclonal tau antibody (tilavonemab, ABBV-8E12); he died of intrahepatic cholangiocarcinoma and gastrointestinal bleeding during the clinical trial. Neuropathological examination demonstrated neuronal loss, gliosis, and widespread deposits of phosphorylated tau in the neurofibrillary tangles, tufted astrocytes, coiled bodies, and threads, which mainly occurred in the inferior olive nucleus, dentate nucleus of the cerebellum, substantia nigra, midbrain tegmentum, subthalamic nuclei, globus pallidus, putamen, and precentral gyrus, confirming typical PSP pathology. Phosphorylated tau was also found to accumulate in Betz cells, Purkinje cells, and pencil fibers in the basal ganglia. In conclusion, no additional changes or pathological modifications, which were expected from immunotherapy targeting tau, were visible in the present case.

Keywords: autopsy; monoclonal antibody; progressive supranuclear palsy; tau; tilavonemab.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal / therapeutic use
  • Autopsy
  • Basal Ganglia / pathology
  • Humans
  • Male
  • Neurofibrillary Tangles / pathology
  • Supranuclear Palsy, Progressive* / pathology
  • tau Proteins / metabolism

Substances

  • Antibodies, Monoclonal
  • tau Proteins