Sporadic Creutzfeldt-Jakob disease (sCJD) can be a consequence of corneal transplants, but the incidence and mechanism remains a mystery. Researchers out of San Francisco, however, believe they’ve developed a method by which optometrists can identify the condition soon after implantation, giving patients more of a fighting chance.
The team analyzed the eyes from 11 cases of pathologically confirmed sCJD and six controls with nonprion diseases. They detected prion-seeding activity in 100% of sCJD eyes, of varying levels. The retina consistently showed the highest seed levels, which in some cases were only slightly lower than in the brain. Within the retina, prion deposits were detected by immunohistochemistry (IHC) in the retinal outer plexiform layer in most cases, but some cases had prion deposits in the inner plexiform layer. Prions were not detected by IHC in any other eye region. The investigators evaluated the eyes using optical coherence tomography and horizontal axial B-scans.
The early detection will benefit the patients themselves, but the researchers add that eye care professionals must be cognizant of how examining these patients will impact their office. “As the early-disease phase of sCJD often includes visual symptoms, patients with sCJD will often have diagnostic assessments performed by an ophthalmologist, potentially contaminating instruments,” the report reads. “As the early-disease phase of sCJD often includes visual symptoms, patients with sCJD will often have diagnostic assessments performed by an ophthalmologist, potentially contaminating instruments.”
|Orru C, Soldau K, Cordano C. Prion Seeds Distribute throughout the Eyes of Sporadic Creutzfeldt-Jakob Disease Patients. mBio. 2018;9(6):e02095-18.