Supranuclear Gaze Abnormality in Sporadic-Creutzfeldt Jacob Disease.

Sporadic Creutzfeldt-Jacob disease (sCJD) usually presents with typical clinical signs. Prodromal symptoms lasting for several weeks are followed by dementia, myoclonus, ataxia and abnormalities of vision (1). Supranuclear gaze palsies accompanying sCJD have rarely been reported. Some of these reported cases were initially diagnosed as progressive supranuclear palsy (PSP) and the diagnosis was corrected only after pathological examination had revealed spongioform encephalopathy (2,3). We aimed to report a patient who presented with supranuclear ophtalmoparesis, rapidly progressive gait ataxia and dementia as his initial manifestations of probable sCJD. A 46-year-old man had presented with a 2 monthhistory of rapidly progressive gait instability, slurred speech and cognitive deterioration. He also reported diplopia and insomnia. The patient was alert but disoriented and his mini mental status examination score was 21. Voluntary vertical eye movements were almost abolished, but could be elicited by doll’s eyes maneuver. In horizontal gaze examination, his smooth pursuit was replaced by microsaccades and his eyes were unable to perform convergence. He had a severe cerebellar dysarthria, impaired rapid alternating movements and severe bilateral dysmetria of upper and lower extremities. He also had a moderate gait ataxia. Neurological examination was otherwise normal. His routine blood chemistry was normal. Cerebrospinal fluid analysis was normal for cell counts, protein and glucose levels, oligoclonal bands, infectious agent and paraneoplastic antibody scanning. However CSF 14-3-3 protein was positive and CSF total tau level was highly increased (1646 pg/ml). Brain MRI revealed bilateral FLAIR and T1 hyperintensities of caudate nuclei, putamina and frontal lobe involving most of the anterior cingulate gyrus and superior frontal gyrus bilaterally, with restricted diffusion (Figure 1). EEG showed decreased amplitudes but no typical periodic sharp waves. He was diagnosed as 91