Hallucinatory palinopsia and paroxysmal oscillopsia as initial manifestations of sporadic Creutzfeldt-Jakob disease: A case study.

BACKGROUND: Heidenhain variant of Cruetzfeldt Jacob Disease is a rare phenotype of the disease. Early and isolated visual symptoms characterize this particular variant of CJD. Other typical symptoms pertaining to muti-axial neurological involvement usually appear in following weeks to months. Commonly reported visual difficulties in Heidenhain variant are visual dimness, restricted field of vision, agnosias and spatial difficulties. We report here a case of Heidenhain variant that presented with very unusual symptoms of palinopsia and oscillopsia.

CASE PRESENTATION: A 62-year-old male patient presented with symptoms of prolonged afterimages following removal of visual stimulus. It was later on accompanied by intermittent sense of unstable visual scene. He underwent surgery in suspicion of cataratcogenous vision loss but with no improvement in symptoms. Additionally he developed symptoms of cerebellar ataxia, cognitive decline and multifocal myoclonus in subsequent weeks. On the basis of suggestive MRI findings in brain, typical EEG changes and a positive result of 14-3-3 protein in CSF, he was eventually diagnosed as sCJD.

CONCLUSION: This case adds to the tally of handful reports of Heidenhain variant CJD in literature, particularly from India. Two atypical initial symptoms, namely hallucinatory palinopsia and paroxysmal oscillopsia were observed in the index case. Possible explanations of such phenomena in CJD have been explored in light of the available studies.