55-year-old man with progressive dementiaTeaching NeuroImages

NeurologyResident and Fellow Section

Vignette

• A 55-year-old African-Canadian man presented with diabetic ketoacidosis

• History of insulin-dependent diabetes mellitus and alcohol abuse

• Progressive cognitive decline (severe impairment of orientation/attention/recall/comprehension/naming) over the previous five years resulting in long-term care placement

• Aside from pigmentary retinopathy, general examination was unremarkable

Parks et al.

Imaging

Parks et al.

Imaging

Parks et al.

Neurodegeneration with brain iron accumulation in aceruloplasminemia

• Ceruloplasmin undetectable, low serum iron, low serum copper, and 10-fold increase in serum ferritin

• Diagnosed with aceruloplasminemia• Iron chelation was not administered given

advanced dementia at presentation

Parks et al.