"면역조직화학적으로 확진된
산발성 크로이츠펠트-야콥병의 임상 특성" |
고상배, "김용선*·정병훈*·김동규
†
·김상윤" |
"서울대학교 의과대학 신경과학교실,신경외과학교실
†
,한림대학교 생명과학연구소*" |
"Clinical Presentation of Immunohistochemicially Proven
Sporadic Creutzfeldt-Jakob Disease" |
"Sang-Bae Ko, M.D., Yong Sun Kim, M.D., Ph.D.*, Byung-Hoon Jeong, M.S.*,
Dong Gyu Kim, M.D., Ph.D.
†
, Sang-Yun Kim, M.D., Ph.D." |
"Department of Neurology and Department of Neurosurgery
†
, College of Medicine, Seoul National University Institute of Environment Life Science, Hallym University*" |
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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 diag-nosis of Creutzfeldt Jakob disease is to ascertain the PrP sc in brain tissue through immunohistochemistry.
J Korean Neurol Assoc 19(5):464~470, 2001
Key Words : Creutzfeldt-Jakob disease, Prion, PrP, Immunohistochemistry" |
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