Creutzfeldt-Jakob disease (CJD), a spongiform encephalopathy, is a rare and fatal condition that affects the brain. It causes brain damage that worsens rapidly over time. We herein report the case of a 67 years old patient without pathological antecedents who was admitted to our department for visual and gait disturbances, coreo-athetotic movements and confusional state, symptomatology that had started about one month before and worsened progressively. The neurological exam pointed out: cortical blindness, coreoathetotic movements left more than right, hyperestesia. Laboratory test results revealed hyperglycemia. All others tests (B12 vitamin, B,C, hepatitis, HIV, FT4, TSH hormones, paraneoplastic and autoimmune markers – NMDA, GABA, VKGT) were physiologically within limits. The cerebrospinal fluid analysis revealed an intense 14-3-3 and TAU protein positivation. The CT scan that was performed did not reveal anything pathological. A cerebral MRI with contrast injection and an EEG were performed. The MRI, which was performed in general anesthesia, revealed: bicerebral cortical cortex hypersignal in diffusion sequence (except the left parietal lobe), with no FLAIR correspondence, without pathological contrast intakes,while EEG highlighted: slow dysrhythmia, delta waves with three phase paroxysmal discharge, Based on these examinations was established the final diagnosis: Probable Sporadic Creutzfeldt Jakob disease. The patient received treatment with valproic acid and clonazepam with no significant improvement.
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