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Brain, Vol. 123, No. 2, 267-276, February 2000
© 2000 Oxford University Press

Semantic dementia with ubiquitin-positive tau-negative inclusion bodies

M. N. Rossor1, T. Revesz2, P. L. Lantos3 and Elizabeth K. Warrington1

1 Dementia Research Group, Department of Clinical Neurology, 2 Neuropathology Department, Institute of Neurology, 3 Department of Neuropathology, Instituteof Psychiatry, London, UK

Correspondence to: Professor M. N. Rossor, Dementia Research Group, The National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK

Three cases are reported with dementia and ubiquitin-positive but tau-negative inclusion bodies. All patients had a semantic dementia and the clinical details of two of these have been published as the first description of a selective semantic memory impairment. The original diagnosis had been of Pick's disease based on frontotemporal atrophy, but re-examination has revealed ubiquitin-positive but tau-negative inclusions as well as neurites in the frontotemporal cortices and ubiquitin-positive, intracytoplasmic inclusions in the granule cells of the dentate fascia. These inclusions are identical to those reported in association with amyotrophic lateral sclerosis (motor neuron disease), but none were seen in brainstem or spinal cord motor neurons.

semantic dementia; frontotemporal dementia; Pick's disease; frontotemporal degeneration; ubiquitin

WAIS = Wechsler Adult Intelligence Scale


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