PANENCEPHALOPATHIC TYPE OF CREUTZFELDT-JAKOB DISEASE WITH PRIMARY EXTENSIVE INVOLVEMENT OF WHITE MATTER

被引：34

作者：

KRUGER, H ^{[1
]}

MEESMANN, C ^{[1
]}

ROHRBACH, E ^{[1
]}

MULLER, J ^{[1
]}

MERTENS, HG ^{[1
]}

机构：

[1] UNIV WURZBURG,INST PATHOL,W-8700 WURZBURG,GERMANY

来源：

EUROPEAN NEUROLOGY | 1990年 / 30卷 / 02期

关键词：

magnetic resonance imaging study; oligoclonal CSF-IgG; panencephalopathic Creutzfeldt-Jakob disease; spongiform encephalopathy;

D O I：

10.1159/000117324

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

The case of a 66-year-old woman with atypical Creutzfeld-Jakob disease (CJD) presents several peculiarities. Pathologic examination confirmed a rare CJD case with primary generalized spongiform changes of the white matter and only moderate, but typical changes of the gray matter. Besides an essentially typical clinical course, the patient developed temporary diplopia and vertical eye movement paralysis. Intoxication had been excluded. Isoelectric focusing revealed oligocloncal CSF-IgG. Magnetic resonance imaging studies showed periventricular accentuated flat and striped hyperintense structures. EEG had CJD-typical periodic 1/s synchronous discharges. This case illustrates for the first time that in panencephalopathic type of CJD the cerebral white matter can be involved primarily and more extensively than the gray matter.

引用

页码：115 / 119

页数：5

共 26 条

[1] CREUTZFELDT-JAKOB DISEASE PRION PROTEINS IN HUMAN BRAINS [J].