What's in a name? That which we call Creutzfeldt-Jakob disease by any other name would be as devastating. And how, do neurologists ever love their names! CJD - also known as "spongiform encephalopathy" and the comically grim "mad cow disease" - is an uncommon but uniformly fatal form of rapidly progressive dementia. In this case, Dr. Makinen takes us through the presenting features, definite diagnosis, and emergent management of the prion-mediated encephalopathy we call CJD. An entity which would, were it not CJD call'd, retain its dear morbidity.