Tau, ubiquitin, and alpha B-crystallin immunohistochemistry define the principal causes of degenerative frontotemporal dementia.

OBJECTIVE

We investigated the use of immunostaining with antibodies to tau, ubiquitin, and alpha B-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-tau 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 alpha B-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.