Creutzfeldt-Jakob disease Heidenhain variant: case report with progressive cognitive decline recorded by videos

Abstract

Context: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative disease. Heidenhain’s variant has isolated visual symptoms that persist even without any cognitive decline for a few weeks. Objective: To report a case of the Heidenhain variant of the CJD with evolution documented. Methods: Case report:A 67-year-old woman was admitted to the emergency room with a report of “seizures and dementia for 1 month”. It all started in August 2020 with a decrease in visual acuity. The son started to record the evolution of the disease in vídeos on his mobile phone. It got worse, with an inability to reach objects and optical apraxia,psychomotor slowing, abnormal repetition of acts and impairment of judgment. MRI of the brain (normal) and EEG was performed, with diffuse slowing of the base activity. It evolved with intermittent myoclonus and abulia. Extensive investigation for encephalitis: normal. Treatment with acyclovir and pulsetherapy with methylprednisolone were performed. Results: new brain MRI: restricted area following the diffusion and T2 / FLAIR hypersignal exclusively cortical involving both posterior hemispheres. Also new EEG: generalized periodic discharges. The 14-3-3 protein was detected in her CSF.The patient died 4 months after the onset of the condition due to infectious complications. Conclusions: The Heidenhain variant of CJD should be considered a differential diagnosis in all patients who have isolated visual complaints, especially if associated with cognitive complaints. The video recording of the patient allows a detailed analysis of the clinical picture and becomes an important diagnostic complementation tool.