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

Paul N. Cooper, MA; Matthew Jackson, MB,BS; Graham Lennox, MA; James Lowe, DM; David M. A. Mann, PhD

Arch Neurol. 1995;52(10):1011-1015.

Abstract
Objective
We investigated the use of immunostaining with antibodies to , ubiquitin, and 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- 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 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.

Author Affiliations
Medical From the Division of Molecular Pathology, Department of Pathology, Manchester University Med'u School (Mr Cooper and Dr Mann), and the Department of Pathology, University of Nottingham Medical School, Queens Medical Centre (Drs Jackson and Lowe and Mr Lennox), England.

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