Tauopathy with hippocampal 4-repeat tau immunoreactive spherical inclusions: a report of three cases.

摘要: Tauopathies are a major group of neurodegenerative proteinopathies characterized by the accumulation abnormal and hyperphosphorylated tau proteins in brain. Tau pathology is as 3R (repeat) or 4R predominant mixed type. Here we report three cases lacking mutations microtubule associated protein (MAPT) gene with unusual pathology. The age at onset duration illness, respectively, were 63 20 years (male), 67 5 (female) 72 (female). clinical presentation was compatible diagnosis progressive supranuclear palsy (PSP) two subjects cognitive decline all subjects. Common neuropathological features included neuronal loss hippocampus dentate gyrus spherical basophilic Pick body-like inclusions showing immunoreactivity, which supported detection predominantly species Western blot examination. In addition, immunoreactive argyrophilic astrocytes amygdala oligodendroglial coiled bodies hippocampal white matter observed. These morphologies appeared combination Alzheimer disease-related subcortical PSP. Together single case literature, our observations on these expand spectrum previously described tauopathies. We suggest that this tauopathy variant might contribute to deficits patients reported here. precise definition clinicopathological relevance pathologies merits further study.