Sporadic Jakob-Creutzfeldt Disease Often Misdiagnosed

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Sporadic Jakob-Creutzfeldt Disease Often Misdiagnosed
Sporadic Jakob-Creutzfeldt Disease Often Misdiagnosed

(HealthDay News) – Patients with sporadic Jakob-Creutzfeldt disease (sCJD) are often misdiagnosed, with only 18% correctly diagnosed at their first assessment, according to a study published online Sept 24 in the Archives of Neurology.

Over a 5.5-year period, Ross W. Paterson, MRCP, from the University of California in San Francisco, and colleagues conducted a retrospective medical record review of 97 patients who had pathologically proven sCJD. The authors determined which medical specialties saw patients with sCJD before the correct diagnosis was made and at what point during the disease history the diagnosis was correctly made.

The researchers found that the most common categories of misdiagnosis were neurodegenerative, autoimmune/paraneoplastic, infection, and toxic/metabolic disorders, with the most common individual diagnoses being viral encephalitis, paraneoplastic disorder, depression, vertigo, Alzheimer's disease, stroke, unspecified dementia, central nervous system vasculitis, peripheral neuropathy, and Hashimoto encephalopathy. Primary care physicians and neurologists most commonly made these misdiagnoses. Almost all of the 18% of patients receiving a correct diagnosis at their first assessment were evaluated by a neurologist. From onset to diagnosis there was a mean time of 7.9 months, which was an average of two-thirds of the way through the disease course.

"When evaluating patients with rapidly progressive dementia with suspected neurodegenerative, autoimmune, infectious, or toxic/metabolic etiology, sCJD should also be included in the differential diagnosis, and appropriate diagnostic tests, such as diffusion brain magnetic resonance imaging, should be considered," the authors write. "Primary care physicians and neurologists need improved training in sCJD diagnosis."

One author disclosed financial ties to the pharmaceutical and medical device industries.

Abstract
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