Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of Fahr's disease presenting "diffuse neurofibrillary tangles with calcification"

Nobuatsu Nomoto, M.D., Hideki Sugimoto, M.D., Hiroaki Iguchi, M.D., Teruyuki Kurihara, M.D. and Nobuo Wakata, M.D.

Fourth Department of Internal Medicine, Toho University School of Medicine

77-year-old woman presented memory disturbance, hallucination, and personality change since the summer of 1988. Laboratory findings revealed normal serum Ca, P, HS-PTH levels and Ellsworth-Howard test was intact. Neuroradiological studies disclosed calcification in the dentate nucleus, putamen, globus pallidus and thalamus. We made a diagnosis of Fahr's disease. During the follow-up period of 13 years, brain MRI showed gradual atrophy in temporal lobes. This case was characterized by the clinical and neuroradiological findings of "diffuse neurofibrillary tangles with calcification: DNTC". We discussed the relations of Fahr's disease and DNTC in the literatures. From our long time observation of this case, we suggest that Fahr's disease includes DNTC.

(CLINICA NEUROL, 42: 745|749, 2002)
key words: presenile dementia, diffuse neurofibrillary tangles with calcification, Fahr's disease, periventricular hyperintensity, leukoaraiosis

(Received: 5-Mar-02)