THREE CASES OF CREUTZFELDT JAKOB DISEASE——a clinicol and pathological analysis
- VernacularTitle:Creutzfeldt-Jakob病3例临床及病理分析
- Author:
Wenfu LU
;
Luning WANG
;
Chuanqian PU
- Publication Type:Journal Article
- Keywords:
Creutzfeldt Jakob disease;
clinic;
pathology
- From:
Medical Journal of Chinese People's Liberation Army
1982;0(01):-
- CountryChina
- Language:Chinese
-
Abstract:
To study the features of sporadic Creutzfeldt Jakob Disease(CJD) in China. Three cases of CJD diagnosed by clinical, electroencephalography (EEG), MRI,14 3 3 brain protein in cerebrospinal fluid (CSF),pathology and immunocytochemistry prion protein (PrP) were analysed. The average age of the onset of CJD was sixty four years.The onset was acute in all three cases. Visual and cerebellar signs appeared comparatively early besides mental and myoclonic symptoms,but signs of pyramidal damage were not obvious relatively. In all the three cases EEG showed periodic sharp wave complexes. Calcification of pallidumes was showed by CT in one case. Brain atrophy was not so obvious in three cases on MRI. One case showed increased signal surrounding both lateral ventricles of the brain on T2 weighted images. Biopsy,narked changes in the frontal lobe in one case,and in the occiptal lobe in another case were observed,with disappearance of nerve cells and extensive astroglial proliferation and spongiform state. Anti PrP immunocytochemistry had detected PrP deposits in the one biopsy,but the 14 3 3 brain protein was negative in CSF. The diagnosis of CJD was made clinically, but the 14 3 3 brain protein was suspicious one time and negative in another test. It suggested that CJD in Chinese patients may consist of the following features: 1. The onset was comparatively early in age. 2. The onset was acute. 3. Visual and cerebellar signs appeared comparatively early besides mental and myoclonic changes,but signs of pyramidal damage were not obvious relatively. 4. Brain atrophy was not remarkable. 5. Farther study with MRI should be carried out. 6. Negative 14 3 3 brain protein test could not rule out CJD.
