DR. DEVIKA BHATTACHARYA MANDLIK

DR. PRIYA SIVA KUMAR

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative spongiform encephalopathy due to infectious agents composed of proteins called prions (PrP) rapidly progressive with 100 % mortality. Only few patients present with visual symptoms initially. A 62 years female presented with complaints of green coloured vision & appearance of insects & hairs in BE for 1 week. BCVA was 6/9. Ocular exam showed defective colour vision & inability to perform central fields. Pupils were brisk, fundus normal. She denied hypertension, diabetes, psychosis, migraine or epilepsy. Patient had impaired cognition ,conscious, disoriented ,along with anterograde memory loss. Gait disturbances, deranged mental functions arouse a suspicion of cortical insult. MRI brain revealed ribbon like plaques in the occipital cortex suggestive of deposition of PrP & triphasic waves in the EEG. Despite the lack of treatment of this incurable disease ,counselling for end of life crises to patients & family is mandatory.