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

      case-report

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          Abstract

          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

          Journal
          ENE
          Eur Neurol
          10.1159/issn.0014-3022
          European Neurology
          S. Karger AG
          0014-3022
          1421-9913
          1990
          1990
          14 February 2008
          : 30
          : 2
          : 115-119
          Affiliations
          aNeurologische Klinik der Universität Würzburg, und bInstitut für Pathologie der Universität Würzburg
          Article
          117324 Eur Neurol 1990;30:115–119
          10.1159/000117324
          2187697
          e0b2a8b5-d72e-45b9-b02c-d7cfff911d66
          © 1990 S. Karger AG, Basel

          Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

          History
          : 02 May 1989
          : 14 September 1989
          Page count
          Pages: 5
          Categories
          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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