Diffuse neurofibrillary tangles with calcification

Diffuse neurofibrillary tangles with calcification (DNTC) is a primary and sporadic presenile dementia that is characterized by temporal and/or frontal atrophy with diffuse neurofibrillary tangles (NFTs) and Fahr-type calcification without senile plaques (SPs). We reviewed clinical symptoms in 21 autopsy-proven DNTC cases in Japan. It is characterized by slowly progressive cortical dementia mimicking Alzheimer's disease (AD) because of the appearance of memory disturbance and disorientation in the early stages, or simulating Pick's disease as a result of the presence of personality changes in the early to middle clinical stages, followed by various psychiatric and neurological symptoms such as Parkinson's symptoms, aphasia, and apraxia. In terms of neuropathology, DNTC is characterized by the appearance of massive NFTs in the cerebral cortex and in Meynert's nucleus and the locus ceruleus/raphe nuclei as well. These sites are similar to those with AD. Differing from AD, SPs were absent, or only a small number were detected. Ultrastructural and immunohistochemical study revealed that NFTs with DNTC were morphologically and biochemically the same with those in AD. Fahr-type calcification is also one of the most characteristic findings; it was found in the basal ganglia and the cerebellum. We conducted elementary analysis of the calcified sites. In addition to calcium and iron, a higher level of lead was detected in comparison with control groups. The etiology should be further investigated.