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KMID : 0364519960080020335
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Dong-A Journal Medicine
1996 Volume.8 No. 2 p.335 ~ p.344
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Two Cases of Creutzfeldt-Jakob disease
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Kim Sang-Woo
Kim Sang-Ho
Kim Jae-Woo
Kim Ki-Uk
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Abstract
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Creutzfeldt-Jakob disease (subacute spongiform encephalopathy) in found throughout the world and presumably caused by a slow infections pathogen. We report two patients who presented progressive dementia, diffuse myoclonus, gait disturbance, cortical blindness and died about 4 and 7 months after onset of symptoms and signs, respectively. These two patients rapidly deteriorated and became comatose. The myoclonus which did not respond to amantadine was rapidly generalized from one side of hand or foot, and appeared in eyeball and other facial muscles, and gradually disappeared at the terminal stage. Video electroencephalography and electromyography showed that diphasic or triphasic sharp waves were synchronized with increased myoclonic movements. Serial EEG revealed progressive slowing of background rhythm and periodic (0.5-1 Hz) high voltage, bilateral diphasic or triphasic sharp wavers and occasional electrically secondary generalization and reduced amplitude in the late sage. CT-guided stereotactic brain biopsy was done in putamen and frontal lobe in one case. Light microscopic findings showed a diffuse small vacuoles in neuropil and a mild degree of microglial cell activation and decreased neuronal population, which were strongly suggestive of spongiform ncephalopathy.
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