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Editorial |

The (Mis)diagnosis of Creutzfeldt-Jakob Disease

Richard J. Caselli, MD
Arch Neurol. 2012;69(12):1554-1555. doi:10.1001/2013.jamaneurol.1.
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I recall, as a young resident in the 1980s, that Creutzfeldt-Jakob disease (CJD) seemed much easier to diagnose than it seems to me now. How can that be? Graying synapses aside, I suspect it reflects the progress that has been made in the past 30 years, particularly in the area of autoimmune encephalopathy. Although CJD at one time seemed to have a monopoly on rapidly progressive dementia, the increasing recognition of autoimmune encephalopathy has made us less confidant about pronouncing the “death sentence.” Patients with autoimmune encephalopathy can be highly responsive to steroids; however, that is not always the case. Paraneoplastic cases typically are not at all responsive and usually end in death. New antibodies continue to be found, making us further wary of the CJD pronouncement only to find, weeks later, that a new antibody not previously sought has been identified. So, increasingly, our attention is focused on not missing reversible causes with somewhat reduced concern for the timely diagnosis of an invariably fatal disease. In this issue of Archives of Neurology, however, Paterson and colleagues1 remind us of perhaps a more traditional perspective.

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Figure. Diffusion-weighted images of anoxic-ischemic encephalopathy in a 17-year-old girl demonstrating a cortical ribbon pattern most prominently in occipital-temporal and paracentral cortices.




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