Dementia Japan 20: 10-16, 2006

Frontotemporal dementia and ubiquitin-positive tau-negative intraneuronal inclusions

Koichi Okamoto

Department of Neurology, Gunma University Graduate School of Medicine

    I reviewed the recent advances of frontotemporal dementia and ubiquitin-positve tau-negative neuronal inclusions. In 1991, we first reported that the inclusions were seen in the extramotor cortices of 7 out of 27 sporadic ALS patients, and then the inclusions were found in the extramotor cortices of all 10 presenile dementia with motor neuron disease. We also reported the ultrastructure of the inclusions and they consisted mainly of granules associated with a few filaments. The inclusions were immunoreactive for vacuole creating protein and p62, however morphogenesis and nature of the inclusions have not yet been known. It is very important to identify of the major protein component of these inclusions.

Address correspondence to Dr. Koichi Okamoto, Department of Neurology, Gunma University Graduate School of Medicine (3-39-22, Shouwamachi, Maebashi, Gunma 371-8511, Japan)