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      Panencephalopathic Type of Creutzfeldt-Jakob Disease with Primary Extensive Involvement of White Matter


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          The case of a 66-year-old woman with atypical Creutzfeldt-Jacob 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 oligoclonal 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.

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          Author and article information

          Eur Neurol
          European Neurology
          S. Karger AG
          14 February 2008
          : 30
          : 2
          : 115-119
          aNeurologische Klinik der Universität Würzburg, und bInstitut für Pathologie der Universität Würzburg
          117324 Eur Neurol 1990;30:115–119
          © 1990 S. Karger AG, Basel

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          : 02 May 1989
          : 14 September 1989
          Page count
          Pages: 5
          Original Paper

          Geriatric medicine,Neurology,Cardiovascular Medicine,Neurosciences,Clinical Psychology & Psychiatry,Public health
          Panencephalopathic Creutzfeldt-Jakob disease,Spongiform encephalopathy,Magnetic resonance imaging study,Oligoclonal CSF-IgG


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