This study provides Class I evidence that quinacrine does not improve survival for people with sCJD when given orally at a dose of 300 mg per day for 2 months.

To identify the misdiagnoses of patients with sporadic Jakob-Creutzfeldt disease (sCJD) during the course of their disease and determine which medical specialties saw patients with sCJD prior to the correct diagnosis being made and at what point in the disease course a correct diagnosis was made.Design: Retrospective medical record review.Setting: A specialty referral center of a tertiary academic medical center.Participants: One hundred sixty-three serial patients over a 5.5-year period who ultimately had pathologically proven sCJD. The study used the subset of 97 patients for whom we had adequate medical records.Main Outcome Measures: Other diagnoses considered in the differential diagnosis and types of medical specialties assessing patients with sCJD.Results: Ninety-seven subjects' records were used in the final analysis. The most common disease categories of misdiagnosis were neurodegenerative, autoimmune/ paraneoplastic, infectious, and toxic/metabolic disor-ders. The most common individual misdiagnoses were viral encephalitis, paraneoplastic disorder, depression, vertigo, Alzheimer disease, stroke, unspecified dementia, central nervous system vasculitis, peripheral neuropathy, and Hashimoto encephalopathy. The physicians who most commonly made these misdiagnoses were primary care physicians and neurologists; in the 18% of patients who were diagnosed correctly at their first assessment, the diagnosis was almost always by a neurologist. The mean time from onset to diagnosis was 7.9 months, an average of two-thirds of the way through their disease course.Conclusions: Diagnosis of sCJD is quite delayed. 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. Primary care physicians and neurologists need improved training in sCJD diagnosis.

Recent cases of prion transmission in humans following transfusions using blood donated by asymptomatic variant Creutzfeldt-Jakob disease (CJD) patients implicate the presence of prion infectivity in peripheral blood. In this study, we examined the levels of the normal, cellular prion protein (PrPC) and the disease-causing isoform (PrPSc) in subpopulations of circulating white blood cells (WBC) from sporadic (s) CJD patients, age-matched neurological controls and healthy donors. Though widely distributed, the highest levels of PrPC were found in a subpopulation of T lymphocytes: ~12,000 PrPC molecules were found per CD4+CD45RA-CD62L- effector memory T helper cell. While platelets expressed low levels of PrPC on their surface, their high abundance in circulation resulted in the majority of PrPC being platelet associated. Using quantitative FACS analysis, we found that neither WBC composition nor the amount of cell-surface PrPC molecules was altered in patients dying of sCJD. Eight different WBC fraction types from the peripheral blood of sCJD patients were assessed for PrPSc. We were unable to find any evidence for PrPSc in purified granulocytes, monocytes, B cells, CD4+ T cells, CD8+ T cells, NK cells, non-classical γδT cells, or platelets. If human WBCs harbor prion infectivity in sCJD patients, then the levels are likely to be low.

No abstract