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Different variants of frontotemporal dementia: a neuropathological and immunohistochemical study |
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| Authors: | M. Bergmann K. Kuchelmeister K. W. Schmid H. A. Kretzschmar R. Schröder |
| Affiliation: | Institute of Clinical Neuropathology, Zentralkrankenhaus Bremen-Ost, Züricher Strasse 40, D-28325 Bremen, Germany, DE Institute of Neuropathology, University of Münster, Münster, Germany, DE Institute of Pathology, University of Münster, Münster, Germany, DE Institute of Neuropathology, University of G?ttingen, G?ttingen, Germany, DE Department of Neuropathology, University of Cologne, Cologne, Germany, DE |
| Abstract: | Histological and immunohistochemical findings in 20 cases of frontotemporal dementias – 8 cases of dementia of frontal lobe type (DFT), 7 cases of Pick’s disease (PD), and 5 cases of motor neuron disease with dementia (MND/D) – are presented. Common features of all three syndromes were: frontotemporal atrophy, involvement of subcortical nuclei, and swollen chromatolytic cells. Ubiquitin (Ub)-positive and tau-negative inclusions in cortical, hippocampal, and motor neurons were found in MND/D and DFT cases, suggesting a common pathogenesis of MND/D and DFT. MND/D showed the same cytoskeletal alterations in motor nuclei as MND without dementia: Bunina bodies and skein-like, Ub-positive inclusions. DFT differed from PD in the preponderance of histopathological changes in upper cortical layers, the sparseness of chromatolytic cells, and the absence of tau-positive Pick bodies (PBs). There were, however, two transitional cases showing Pick-type histology but no PBs, thus linking DFT and PD. PBs expressed chromogranin B and secretoneurin strongly, but chromogranin A only weakly. They were negative for the 70-kDa heat-shock protein, metallothionein, and glutathione-S-transferase. Received: 6 November 1995 / Revised: 12 January 1996 / Revised, accepted: 2 February 1996 |
| Keywords: | Frontotemporal dementia Dementia of frontal lobe type Pick’ s disease Motor neuron disease with dementia Pick bodies |
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