Changes of diffuse neurofibrillary tangles with calcification (DNTC) in a woman without evidence of dementia.

Diffuse neurofibrillary tangles with calcification (Kosaka–Shibayama disease) in Japan

“…Langlois et al . also described a woman without evidence of dementia . Her brain weighed 1300 g and NFT were numerous in the hippocampus and parahippocampal cortex.…”

“…A literature search for DNTC in other countries revealed only four reports, of which only two were autopsies . The autopsy described by Tariska was the second such report in the world, and that of Langlois was the first description of an autopsy of a patient with early DNTC . None of these patients had familial disease.…”

“…We searched the published work for all reports regarding DNTC, including autopsy studies, between 1965 and 2012 using PubMed, Medline, JDREAM III and Igaku Chuo Zasshi. To the best of our knowledge, 26 autopsies of individuals with DNTC have been reported in Japan, and only two autopsies in Europe and the USA have been reported . Here, we review all autopsies reported in Japan, and propose clinical diagnostic criteria for DNTC.…”

Clinicopathological study of diffuse neurofibrillary tangles with calcification

“…Meanwhile, there were some reports of DNTC without sufficient clinical or pathological assessment [4,45,46]. Cases of Fahr's syndrome with neuropsychological deficits and neuropsychiatric features have also been reported based on radiological findings [3,47,48].…”

Distribution of neurofibrillary tangles in diffuse neurofibrillary tangles with calcification

“…Until now, more than 20 cases of DNTC have been reported in Japan, but only two cases 17,18 in American and European countries. Now, this disease is also called Kosaka–Shibayama disease 15 …”