Ubiquitin, and B-Crystallin Immunohistochemistry Define the Principal Causes of Degenerative Frontotemporal Dementia

Abstract

Objective: We investigated the use of immunostaining with antibodies to τ, ubiquitin, and αB-crystallin in defining a protocol for the staged neuropathologic examination of brains from patients with a progressive frontotemporal dementia. Design: Brains obtained from 50 patients dying with the clinical diagnosis of frontotemporal dementia were examined histopathologically to define pathologic distinctions. Setting: Two university hospital neuropathology departments. Results: Anti-τ immunostaining defined corticobasal degeneration, Alzheimer's disease, and Pick's disease; antiubiquitin defined motor neuron disease with dementia. The remaining brains have frontal lobe degeneration: the use of αB-crystallin immunostaining, on these, to detect ballooned neurons may help to define two groups of patients, one of which we believe may represent a variant of Pick's disease. Conclusion: These findings indicate that immunostaining with these antibodies is essential for the evaluation of frontal dementia.