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

ABSTRACT

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.