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Recently Kosaka reviewed 16 cases of slowly progressive presenile cortical dementia thought to be a clinicopathological entity with pathological features characterised by circumscribed lobar atrophy, diffuse neurofibrillary tangles, and calcification of the Fahr type and named diffuse neurofibrillary tangles with calcification.1 However, the pathogenesis and biochemical findings of this new entity are not known in detail. Herein, we report on a patient with presenile dementia, intracranial calcification, and M-proteinaemia, which may play some part in the development of intracranial calcification with dementia.
A 66 year old man was admitted with dementia, which had been apparent since about 60 years of age. At the age of 62 he started to lose spontaneity and at the age of 66 gait disturbance occurred. In addition he showed slurred dysarthria, mild muscle rigidity, bilateral pyramidal signs, and mild …