Diffuse neurofibrillary tangles with calcification / 中华老年医学杂志

Diffuse neurofibrillary tangles with calcification(DNTC)is a rare neurodegenerative disease and belongs to the category of tauopathies.The main characteristics of DNTC include presenile dementia, symmetrical bilateral calcification in the basal ganglia and cerebral dentate nuclei, and bilateral atrophy of the temporal lobes.At present, the majority of reported cases with DNTC were from Japan, with only a few cases from other countries.Although DNTC is a pathological diagnosis based on autopsy, ante-mortem diagnosis for DNTC can be achieved based on clinical features and presentation of brain tau protein shown on positron emission computed tomography.The clinical characteristics, pathological changes and diagnosis criteria of DNTC are reviewed in this paper.

Research advances in diffuse neurofibrillary tangles with calcification (Kosaka-Shibayama disease) / 中华神经科杂志

Diffuse neurofibrillary tangles with calcification (DNTC) is a type of presenile dementia. The clinical features are temporal and/or frontal and/or extrapyramidal symptoms. Memory impairment which might be less prominent in early stages of disease becomes evident with progression; The imagings are featured with bilateral basal ganglia and/or cerebellar dentate calcification (Fahr-type calcification) and/or localized atrophy of temporal or temporofrontal lobes; The pathological features are diffuse neurofibrillary tangles without large number of Aß amyloid plaques (characteristic of Alzheimer′s disease) and without Pick body (characteristic of Pick disease), and calcium deposition is similar to Fahr′s disease in sites but minor in degree. Till now, most case reports are from Japan. It is speculated that there are a large number of misdiagnosis in other countries. Criteria for clinical diagnosis of DNTC was proposed by Iwai K(1995), Kosaka H(2001), Ukai K and Kosaka K (2016, 2017) successively, which can facilitate the diagnosis.

Research advances in diffuse neurofibrillary tangles with calcification (Kosaka?Shibayama disease) / 中华神经科杂志

Diffuse neurofibrillary tangles with calcification (DNTC) is a type of presenile dementia. The clinical features are temporal and/or frontal and/or extrapyramidal symptoms. Memory impairment which might be less prominent in early stages of disease becomes evident with progression; The imagings are featured with bilateral basal ganglia and/or cerebellar dentate calcification (Fahr?type calcification) and/or localized atrophy of temporal or temporofrontal lobes; The pathological features are diffuse neurofibrillary tangles without large number of A?amyloid plaques (characteristic of Alzheimer′s disease) and without Pick body (characteristic of Pick disease), and calcium deposition is similar to Fahr′s disease in sites but minor in degree. Till now, most case reports are from Japan. It is speculated that there are a large number of misdiagnosis in other countries. Criteria for clinical diagnosis of DNTC was proposed by Iwai K(1995), Kosaka H(2001), Ukai K and Kosaka K (2016, 2017) successively, which can facilitate the diagnosis.