A 70-year-old man with extrapyramidal symptoms, dementia and hemosiderosis.

摘要

A 70-year-old man presented with an 8-year history of cognitive decline. Initial presenting signs included mild memory loss, behavioral disturbances, and transient disorientation. He had frontal release signs, including severe confusion, hallucinations and bilateral grasping, pyramidal signs, facial dyskinesia and dysmetria. Initial "mini mental status" (MMS) was quoted as 25/30. Laboratory investigations showed undetectable ceruloplasmin in serum, increased ferritin (1777 mug/L-nl 30-300) and a slight decrease of serum iron (11.8.JJ,mol/L-nl 12-30) and copper (0.06mg/L-nl 0.8-1.4). Fundus oculi was normal; there was neither Kayser-Fleischer ring nor retinal abnormality. There were no signs of diabetes mellitus. A magnetic resonance image (MRI) revealed bilateral increased signal intensities of deep nuclei on Tl weighted that were more marked on T2 weighted sequences. The abnormalities involved predominandy the putamen, caudate nucleus, pulvinar, and dentate nucleus.