RESUMO
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.
Assuntos
Paralisia Supranuclear Progressiva , Masculino , Humanos , Paralisia Supranuclear Progressiva/patologia , Anticorpos Monoclonais/uso terapêutico , Autopsia , Gânglios da Base/patologia , Emaranhados Neurofibrilares/patologia , Proteínas tau/metabolismoRESUMO
A 60-year-old, right-handed woman was admitted to our hospital for amnesia as the only neurological abnormal findings following the autonomic symptoms and transient episodes of loss of awareness. EEG during the amnesia showed rhythmic alpha activity arising from the left mid-temporal region. Although this ictal activity showed evolution in the frequency and amplitude, the location was limited in the bilateral temporal areas. After the EEG evaluation, her amnesia was resolved immediately, suggesting that her presentation was transient epileptic amnesia (TEA). Meanwhile, given the clinical course and MRI findings (high intensity in the bilateral mesial temporal areas, more on the left), she was diagnosed with non-herpetic limbic encephalitis and treated with steroid and anti-epileptic drugs, leading to the positive outcome. The ictal EEG findings during TEA as the one of the presentation in acute phase of non-herpetic limbic encephalitis may contribute to further investigation of underlying mechanism of TEA.