title: Heidenhain variant of Creutzfeldt-Jakob disease in a patient who had bovine bioprosthetic valve implantation.
abstract: Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder characterized by rapidly progressing dementia, general neurologic deterioration, and death. When the leading symptoms are visual disturbances, it is termed as the Heidenhain variant of CJD (HvCJD). CJD was reported following prion-contaminated pericardium transplants but never after bovine bioprosthetic cardiac valve. In this case report, we describe HvCJD in a patient who had a bovine bioprosthetic cardiac valve implant. An 82-year-old-woman was referred to neuro-ophthalmology clinic for unexplained visual loss that started 1 month previously. Medical history included aortic valve replacement with bovine bioprosthetic valve. On examination, best-corrected visual acuity was 20/120 in the right eye and 20/200 in the left eye; otherwise, the eye examination was normal. Humphrey visual fields revealed complete right homonymous hemianopsia. Magnetic resonance imaging (MRI) demonstrated nonspecific white matter changes. A week later, she was hospitalized due to memory impairment; repeated MRI and total body computed tomography scan showed no significant findings. Electroencephalography recordings and extremely elevated cerebrospinal fluid tau protein were compatible with CJD. The patient died 3 weeks later; autopsy was not performed. The patient had HvCJD. Ophthalmologists being first to see these patients should be aware of this diagnosis. Contaminated bovine bioprosthetic valve might be another source for prion disease. Further research is required to establish this issue.
authors.affiliation: Department of Ophthalmology, Baruch Padeh Medical Center, Poriya, Israel.
authors.forename: Jehard
authors.initials: J
authors.lastname: Hashoul
authors.affiliation: Department of Internal Medicine, Emek Medical Center, Afula, Israel.
authors.forename: Waleed
authors.initials: W
authors.lastname: Saliba
authors.affiliation: Department of Neurology, Emek Medical Center, Afula, Israel.
authors.forename: Irina
authors.initials: I
authors.lastname: Bloch
authors.affiliation: Department of Ophthalmology, Baruch Padeh Medical Center, Poriya, Israel.
authors.forename: Haneen
authors.initials: H
authors.lastname: Jabaly-Habib
mesh_terms: D000369:Aged, 80 and over; D000818:Animals; D001021:Aortic Valve / Q000601:surgery*; D001705:Bioprosthesis / Q000009:adverse effects*; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome; D004569:Electroencephalography; D005260:Female; D006349:Heart Valve Diseases / Q000601:surgery*; D006350:Heart Valve Prosthesis / Q000009:adverse effects*; D006801:Humans; D008279:Magnetic Resonance Imaging; D014057:Tomography, X-Ray Computed; D014786:Vision Disorders / Q000175:diagnosis* / Q000209:etiology