| Autor: |
Pastuszak Ż; Department of Neurology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland., Tomczykiewicz K; Department of Neurology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland., Stępień A; Department of Neurology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland., Piusińska-Macoch R; Department of Neurology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland., Klimczuk J; Department of Neurology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland., Rolewska A; Department of Neurology, Specialized Regional Hospital, Ciechanów, Poland., Galbarczyk D; Department of Neurology, Specialized Regional Hospital, Ciechanów, Poland. |
| Abstrakt: |
Creutzfeldt-Jakob disease (CJD) is a rare syndrome of central nervous system caused by infectious protein called prion. There are four types of CJD: sporadic (sCJD), familial (fCJD), jatrogenic (jCJD) and variant (vCJD). The most frequent symptoms are rapidly progressing dementia, mioclonias, akinetic mutism and signs of cerebellum dysfunction. In sCJD, MRI often shows high signal intensity in the putamen and caudate nucleus on T2-weighted images while in vCJD pulvinar sign is often observed. 70% patients with CJD often has characteristic generalized periodic sharp wave pattern in electroencephalography. In case of 90% patients with CJD 14-3-3 protein is present in cerebrospinal fluid. Neuropathological studies play an important role in disease diagnosis. CJD incidence is 0.5-1 on 1000000 people but some cases can be undiagnosed. Presented study is a description of woman with sCJD confirmed with histopathological study. Since childhood patient had psychotic symptoms and behavior disturbances. Patient wasn't diagnosed due to this symptoms. Few months before admission to hospital her condition was getting worse. Symptoms of cerebellum, pyramidal and extrapyramidal system occurred. In cerebrospinal fluid 14-3-3 protein was detected. In EEG and MRI changes specific for sCJD was observed. After three months patient died. |
