Diffuse neurofibrillary tangles with calcification (DNTC): A rare form of tauopathy

Diffuse neurofibrillary tangles with calcification (DNTC) is a rare form of tanglepredominant dementia. The main clinical symptoms are slowly progressive cortical dementia, showing chiefly memory disturbance. Psychiatric and neurological symptoms, including personality change, aphasia, oral tendency, and parkinsonism, are also observed. Fahr-type calcification, easily detected by CT scan, is a characteristic finding that is useful for discriminating DNTC from other causes of dementia. The brain of DNTC exhibits a circumscribed temporal or temporo-frontal lobar atrophy, a distribution of atrophy that is more similar to that in Pick’s disease rather than Alzheimer’s disease. However, histopathologically, numerous neurofibrillary tangles (NFTs), whose distribution is similar to that in Alzheimer’s disease, are the most prominent histopathological feature, while there are no or only a few senile plaques (SPs), even using immunostaining with an anti-Aβ antibody. In this paper, we present representative cases of DNTC, and review the clinical and pathological features of DNTC cases reported previously.