Sporadic Parkinsonism-Dementia Complex of Rhode Island with Unusually Abundant Glial TDP-43 Immunoreactivity

Background: Parkinson's disease can overlap with other forms of neurodegeneration; however its co-occurrence with both frontotemporal lobar degeneration and motor neuron disease is distinctly rare although characteristic of Parkinsonism-Dementia Complex of Guam (PDCG). Materials and methods:Herein we report a case of a 69-year-old man from Rhode Island whose insidious developments of Parkinson's and motor neuron diseases were followed by a rapidly progressive cognitive decline.Results: The postmortem neuropathological findings were highly reminiscent of PDCG but accompanied by an unusual abundance of TDP-43 immunoreactivity in glia rather than neurons.Conclusion: This case highlights the potential contributions of glial cell dysfunction and TDP-43 pathology in the pathogenesis of neurodegeneration.