2022 Volume 34 Issue 4 Pages 391-398
Diffuse neurofibrillary tangles with calcification (DNTC) is a type of non-Alzheimer’s dementia that belongs to the tauopathy disease group. There have been few reports of DNTC worldwide and less than 30 autopsy and clinical reports in Japan. The bilateral obvious calcification in the basal ganglia of DNTC, are similar to those of idiopathic basal ganglia calcification (IBGC). Differential diagnosis between DNTC and IBGC is thus difficult. We report a male patient with DNTC onset in his 50s. He first visited our clinical department in X year and was aware of forgetfulness since X-1 year. Examinations showed mild forgetfulness but no other neuropsychiatric symptoms. Simple computed tomography (CT) showed marked calcification in the bilateral basal ganglia and dentate nuclei of the cerebellum, and single-photon emission computed tomography (SPECT) showed decreased blood flow with frontal predominance. Neuropsychological examination revealed mild cognitive impairment and mild decline in frontal lobe function according to Wechsler Memory Scale-Revised (WMS-R); however, the Hasegawa Dementia Scale-Revised (HDS-R) score was 30/30. This was a solitary case. Based on the test results and clinical findings, we diagnosed early DNTC with mild memory disability. The course of the disease has progressed slowly with mild deterioration WMS-R in X+2 years; he is still able to continue working with the strong support of his colleague.
