Development of Novel Prion Disease Diagnostic

PROJECT SUMMARY Prion diseases are a class of neurological disorders similar to Alzheimer s disease that are fataltransmissibleand affect both humans and animalsThe most common human prion diseaseCreutzfeldt Jakob diseaseCJDis sometimes called the andquot great mimickerandquotbecause it causes symptoms that occur in many other neurological diseases like Alzheimer s DiseaseSimilar to Alzheimer s diseaseearly and accurate testing of CJD is necessary to distinguish this untreatable disease from treatable rapidly progressive dementiasCommon symptoms of CJDlike Alzheimer s diseaseinclude behavioral and personality changesconfusion and memory problemslack of coordinationand visual disturbancesInterestinglyrecent reports have indicated prion proteins in post mortem retinal tissue of CJD patients similar to reports in the literature in Alzheimer s Disease patientsAmydis is developing an innovative new approach to diagnosing amyloid associated diseases like Alzheimer s disease and CJD by way of an eye examThe proposed research will result in a noninvasive screening test for detecting prions in the eyeas a window to the brainfor early detection of CJD an analogous approach to our initial program in Alzheimer s diseaseIn additionthis test will be utilized by hospitals to reduce the potential transmission of the disease to other patients like patients with Alzheimer s Disease undergoing neurological testsAmydis has designed its technology to use existing ophthalmic instrumentation thereby allowing for an easy transition into commercial practice by physicians Project Narrative Creutzfeldt Jakob DiseaseCJDis characterized by the deposition of insoluble protein aggregates in the brain that are referred to as amyloidsRecentlythese amyloids have been identified in retinal tissue in diseased individualsThe proposed research aims to advance a non invasivediagnostic to image amyloid deposits through the eye