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

Psychiatry Clin Neurosci. 2016 Mar;70(3):131-40. doi: 10.1111/pcn.12334. Epub 2015 Aug 19.

Abstract

Diffuse neurofibrillary tangles with calcification (DNTC) is a rare, pre-senile type of dementia. The term 'DNTC' was initially proposed by Kosaka in 1994. Although 26 autopsies and 21 clinical patients with DNTC have been described in Japan to date, DNTC has rarely been reported in the European and North American published work. We speculate that DNTC has been overlooked in other countries. Herein, we review all known reports of DNTC in Japan and propose clinical diagnostic criteria for DNTC.

Keywords: Fahr's disease; Kosaka-Shibayama disease; clinical diagnostic criteria; diffuse neurofibrillary tangles with calcification; pre-senile dementia.

Publication types

  • Review

MeSH terms

  • Diffuse Neurofibrillary Tangles with Calcification / diagnosis*
  • Diffuse Neurofibrillary Tangles with Calcification / diagnostic imaging
  • Diffuse Neurofibrillary Tangles with Calcification / epidemiology*
  • Humans
  • Japan / epidemiology