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Diffusion-weighted (DWI) magnetic resonance imaging (MRI) is a highly sensitive and specific test for diagnosis of sporadic Jakob-Creutzfeldt disease (sCJD); however, the neuropathologic origin of DWI signal abnormalities including other clinical features has not been well defined. We describe a case of sCJD with brain MRI taken 15 days before death, which(More)
With the discovery of the prion protein (PrP), immunodiagnostic procedures were applied to diagnose Creutzfeldt-Jakob disease (CJD). Before development of the conformation-dependent immunoassay (CDI), all immunoassays for the disease-causing PrP isoform (PrPSc) used limited proteolysis to digest the precursor cellular PrP (PrPC). Because the CDI is the only(More)
In prion-infected mice, both the Notch-1 intracellular domain transcription factor (NICD) and the disease-causing prion protein (PrP(Sc)) increase in the brain preceding dendritic atrophy and loss. Because the drug LY411575 inhibits the gamma-secretase-catalyzed cleavage of Notch-1 that produces NICD, we asked whether this gamma-secretase inhibitor (GSI)(More)
Human prion diseases can be caused by mutations in the prion protein gene PRNP. Prion disease with mutations at codon 188 has been reported in 6 cases, but only 1 had the T188R mutation and it was not pathologically confirmed. We report the clinical, neuropsychologic, imaging, genetic, and neuropathologic features of a patient with familial(More)
551 Background: T1abN0M0 BC has a low recurrence risk. In contrast, HER2+ BC has an increased risk of local/distant recurrence. In small case series of T1abN0M0 HER2+ BCs from tertiary referral centers, recurrence rates have ranged from 8-23%. Data on sites of recurrence have been limited. While adjuvant chemotherapy plus trastuzumab provides a relative(More)
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