The Heidenhain Variant of Creutzfeldt-Jakob Disease: The New Zealand Experience 1997-2016

Creutzfeldt-Jakob Disease (CJD) is a transmissible spongiform encephalopathy resulting in a progressive dementia with associated; neurological features. The Heidenhain variant produces a rapid disease course with prominent early visual symptoms which may precede dementia or other neurologic signs. We identified Heidenhain cases retrospectively from the New Zealand CJD Registry (1997 to 2016). Clinical features including neuro-ophthalmic and neurologic findings, electroencephalograms (EEG), magnetic resonance images (MRI), cerebrospinal fluid (CSF) samples, and neuropathological studies were evaluated if available.