Clinical Presentation of Immunohistochemicially Proven Sporadic Creutzfeldt-Jakob Disease

ABSTRACT

BACKGROUND: Creutzfeldt-Jakob disease is a rapidly progressive, ultimately lethal dementing illness caused by an infectious protein named prion. We present clinical characterisitics, laboratory findings and natural courses of immuno-histochemicially proven sporadic CJD patients. METHODS: The subjects consisted of 5 patients who were confirmed by brain biopsy with immunohistochemistry. We analyzed prodromal symptoms, clinical characteristics, disease duration and laboratory findings retrospectively. RESULTS: The mean age of the patients was 60.2+/-5.8. The prodromal symptoms were variable including dizziness, ataxia, vertigo, imbalance, headache, visual hallucination, visual blurring and ble-pharospasm. The EEG findings showed periodic sharp and wave complexes in all of the patients. MRI findings of 3 patients were high signal intensity in bilateral basal ganglia on T2 weighted and FLAIR images, and DWI showed high signal intensity in temporo-occipital lobe in a patient of Heidenhain variant. CONCLUSIONS: The prodromal symptoms and laboratory findings are not so different from previous report. And we suggest that the only way to confirm the diagnosis of Creutzfeldt Jakob disease is to ascertain the PrP sc in brain tissue through immunohistochemistry. (J Korean Neurol Assoc 19(5):464~470, 2001)