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Unusual features of Creutzfeldt–Jakob disease followed-up in a memory clinic

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Abstract

Sporadic Creutzfeldt–Jakob disease (sCJD) generally manifests itself by cognitive or rapidly progressive motor symptoms. An atypical onset or an unusual evolution may delay the diagnosis. Among patients with a confirmed diagnosis of sCJD following a post-mortem neuropathological examination at the Neuropathology Centre of Lille, France, those who had presented with atypical cognitive disorders at onset were included in the study. Four patients were included. The first patient (64-years-old) presented early language disorders, later accompanied by apathy and behavioral disorders. The prolonged course suggested a diagnosis of progressive primary aphasia. The second patient (68-years-old) presented with aphasia, apraxia, and ataxia of the right upper limb with parkinsonian syndrome, suggesting corticobasal degeneration. In the two last patients (58- and 61-years-old), the onset was marked by an anxiety–depression syndrome, falls, visual hallucinations, extra-pyramidal syndrome, and fluctuating cognitive decline. The diagnosis raised was probable Lewy body dementia. The 14.3.3 protein was found in two of the four cases. The clinical elements found may initially suggest focal atrophy or Lewy body dementia. A very rapid clinical deterioration generally suggests sCJD, but in the last case, the evolution was particularly slow. The diagnosis of sCJD must be considered in cases of rapid-onset dementia, even if all of the clinical criteria are not present. The detection of the 14.3.3 protein and multifold increase in total-Tau with normal or slightly increased phosphorylated-Tau in the CSF are additional arguments to reinforce the diagnosis. The post-mortem neuropathological examination is important to confirm the diagnosis.

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Acknowledgments

We thank Philip Bastable for reviewing the English version of this manuscript.

Conflicts of interest

The authors declare that they have no conflicts of interest.

Ethical standard

This study has been approved by the appropriate ethics committee and has therefore been performed in the accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

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Authors and Affiliations

  1. Centre Mémoire de Ressources et de Recherches, Centre National de référence, Université de Lille-Nord de France, Maladie d’Alzheimer du sujet jeune, Labex DISTALZ, CHU, 59000, Lille, France

    Agnès Jacquin, Vincent Deramecourt & Florence Pasquier

  2. Centre Mémoire de Ressources et de Recherches, Centre Hospitalier Universitaire, Université de Bourgogne, Dijon, France

    Agnès Jacquin

  3. Centre d’anatomopathologie, Université de Lille-Nord de France, 59000, Lille, France

    Vincent Deramecourt & Claude-Alain Maurage

  4. Centre Mémoire de Ressources et de Recherches de Champagne-Ardennes, Centre Hospitalier Universitaire de Reims, Reims, France

    Serge Bakchine

Authors
  1. Agnès Jacquin
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  2. Vincent Deramecourt
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  3. Serge Bakchine
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  4. Claude-Alain Maurage
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  5. Florence Pasquier
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Correspondence to Agnès Jacquin.

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Jacquin, A., Deramecourt, V., Bakchine, S. et al. Unusual features of Creutzfeldt–Jakob disease followed-up in a memory clinic. J Neurol 261, 696–701 (2014). https://doi.org/10.1007/s00415-014-7246-6

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  • DOI: https://doi.org/10.1007/s00415-014-7246-6

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