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Neurology. 2013 Nov 12;81(20):e151-2. doi: 10.1212/

Teaching NeuroImages: neurodegeneration with brain iron accumulation in aceruloplasminemia.

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From the Division of Neurology, Department of Medicine (N.E.P.), and the Division of Neuroradiology, Department of Diagnostic Radiology (R.A.V.), Dalhousie University, Halifax, Nova Scotia, Canada; and the Department of Neurology (J.J.M.), Yale University, New Haven, CT.


A 55-year-old African Canadian man with insulin-dependent diabetes mellitus and alcohol abuse presented with diabetic ketoacidosis. Progressive cognitive decline over the previous 5 years resulted in long-term care placement. Aside from pigmentary retinopathy, general examination was unremarkable. MRI demonstrated iron accumulation in the brain (figure 1) and liver (figure 2A). Ceruloplasmin, a ferroxidase enzyme important in iron homeostasis, was undetectable and associated with low serum iron, low serum copper, and 10-fold increase in serum ferritin. Liver biopsy confirmed increased hepatocyte iron storage (figure 2B). Aceruloplasminemia was diagnosed.(1,2) Iron chelation was not administered given advanced dementia at presentation.

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