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Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies

BACKGROUND: Differential diagnosis of severe progressive dementia includes a wide spectrum of inflammatory and neurodegenerative diseases. Particularly challenging is the differentiation of potentially treatable autoimmune encephalitis and Creutzfeldt-Jakob disease. Such a coincidence may indeed com...

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Autores principales: Zuhorn, Frédéric, Hübenthal, Almut, Rogalewski, Andreas, Dogan Onugoren, Müjgan, Glatzel, Markus, Bien, Christian G, Schäbitz, Wolf-Rüdiger
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4255969/
https://www.ncbi.nlm.nih.gov/pubmed/25434587
http://dx.doi.org/10.1186/s12883-014-0227-7
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author Zuhorn, Frédéric
Hübenthal, Almut
Rogalewski, Andreas
Dogan Onugoren, Müjgan
Glatzel, Markus
Bien, Christian G
Schäbitz, Wolf-Rüdiger
author_facet Zuhorn, Frédéric
Hübenthal, Almut
Rogalewski, Andreas
Dogan Onugoren, Müjgan
Glatzel, Markus
Bien, Christian G
Schäbitz, Wolf-Rüdiger
author_sort Zuhorn, Frédéric
collection PubMed
description BACKGROUND: Differential diagnosis of severe progressive dementia includes a wide spectrum of inflammatory and neurodegenerative diseases. Particularly challenging is the differentiation of potentially treatable autoimmune encephalitis and Creutzfeldt-Jakob disease. Such a coincidence may indeed complicate the correct diagnosis and influence subsequent treatment. CASE PRESENTATION: A 75-year-old woman was admitted due to rapid progressive cognitive impairment. Her husband observed a temporal disorientation and confusion. The initial neurological examination and an extensive neuropsychological evaluation showed significant impairments in almost all tested cognitive domains. All other neurological functions including motor, sensory and coordinative function were intact. Initial diagnostics included EEG, MRI and lumbar puncture with unspecific results. Complementary blood testing revealed a positive result for antineural antibodies to Contactin-associated protein 2 (CASPR2) and the patient received treatment for CASPR2 autoimmune encephalitis. Further symptoms and results, including 14-3-3 proteins, led to suspected Creutzfeldt-Jakob disease. The postmortem examination supported the diagnosis of a definitive Creutzfeldt-Jakob disease. CONCLUSION: One could argue that global screening for antineural antibodies may lead to a false diagnosis triggering intense and potentially dangerous procedures. We believe, however, that potentially treatable causes of dementia should aggressively sought out and subsequently treated in an attempt to curtail the course of disease and ultimately reduce the rate of mortality.
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spelling pubmed-42559692014-12-05 Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies Zuhorn, Frédéric Hübenthal, Almut Rogalewski, Andreas Dogan Onugoren, Müjgan Glatzel, Markus Bien, Christian G Schäbitz, Wolf-Rüdiger BMC Neurol Case Report BACKGROUND: Differential diagnosis of severe progressive dementia includes a wide spectrum of inflammatory and neurodegenerative diseases. Particularly challenging is the differentiation of potentially treatable autoimmune encephalitis and Creutzfeldt-Jakob disease. Such a coincidence may indeed complicate the correct diagnosis and influence subsequent treatment. CASE PRESENTATION: A 75-year-old woman was admitted due to rapid progressive cognitive impairment. Her husband observed a temporal disorientation and confusion. The initial neurological examination and an extensive neuropsychological evaluation showed significant impairments in almost all tested cognitive domains. All other neurological functions including motor, sensory and coordinative function were intact. Initial diagnostics included EEG, MRI and lumbar puncture with unspecific results. Complementary blood testing revealed a positive result for antineural antibodies to Contactin-associated protein 2 (CASPR2) and the patient received treatment for CASPR2 autoimmune encephalitis. Further symptoms and results, including 14-3-3 proteins, led to suspected Creutzfeldt-Jakob disease. The postmortem examination supported the diagnosis of a definitive Creutzfeldt-Jakob disease. CONCLUSION: One could argue that global screening for antineural antibodies may lead to a false diagnosis triggering intense and potentially dangerous procedures. We believe, however, that potentially treatable causes of dementia should aggressively sought out and subsequently treated in an attempt to curtail the course of disease and ultimately reduce the rate of mortality. BioMed Central 2014-11-30 /pmc/articles/PMC4255969/ /pubmed/25434587 http://dx.doi.org/10.1186/s12883-014-0227-7 Text en © Zuhorn et al.; licensee BioMed Central Ltd. 2014 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Zuhorn, Frédéric
Hübenthal, Almut
Rogalewski, Andreas
Dogan Onugoren, Müjgan
Glatzel, Markus
Bien, Christian G
Schäbitz, Wolf-Rüdiger
Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
title Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
title_full Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
title_fullStr Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
title_full_unstemmed Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
title_short Creutzfeldt-Jakob disease mimicking autoimmune encephalitis with CASPR2 antibodies
title_sort creutzfeldt-jakob disease mimicking autoimmune encephalitis with caspr2 antibodies
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4255969/
https://www.ncbi.nlm.nih.gov/pubmed/25434587
http://dx.doi.org/10.1186/s12883-014-0227-7
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