Ubiquitin-positive tau-negative intraneuronal inclusions in dementia with motor neuron disease
The 50th Anniversary of Japanese Society of Neuropathology
Article first published online: 24 MAY 2010
© 2010 Japanese Society of Neuropathology
Volume 30, Issue 5, pages 486–489, October 2010
How to Cite
Okamoto, K. (2010), Ubiquitin-positive tau-negative intraneuronal inclusions in dementia with motor neuron disease. Neuropathology, 30: 486–489. doi: 10.1111/j.1440-1789.2010.01121.x
- Issue published online: 24 MAY 2010
- Article first published online: 24 MAY 2010
- Received 10 February 2010; revised 9 March 2010 and accepted 10 March 2010; published online 24 May 2010.
- amyotrophic lateral sclerosis;
- frontotemporal lobar degeneration;
- motor neuron disease;
We first reported ubiquitin-positive tau-negative intraneuronal inclusions in the hippocampal granular cell layer and entorhinal cortices in patients with amyotrophic lateral sclerosis (ALS). We then found that those inclusions occur frequently in patients with presenile dementia and motor neuron disease. The ultrastructure of the inclusions consists mainly of granules with a few filaments. In 2006, TDP-43 was identified as a major component of the inclusions specific for frontotemporal lobar degeneration and ALS. Here, we review the current knowledge regarding ubiquitin-positive tau-negative intraneuronal inclusions.