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Prion Diseases pp 153-159 | Cite as

Clinical and differential diagnosis of Creutzfeldt-Jakob disease

  • S. Poser
  • I. Zerr
  • A. Schroeter
  • M. Otto
  • A. Giese
  • B. J. Steinhoff
  • H. A. Kretzschmar
Chapter
Part of the Archives of Virology. Supplementa book series (ARCHIVES SUPPL, volume 16)

Summary

Until recently, the clinical diagnosis of CJD relied mainly on three criteria. These include patient history (rapidly progressive dementia), neurological findings (ataxia, pyramidal/extrapyramidal signs, myoclonus, akinetic mutism) and typical electroencephalographic (EEG) findings. These criteria are fulfilled in typical cases. The occurrence or increase of certain proteins in cerebrospinal fluid (CSF; 14-3-3, neuron-specific enolase) now provide important adjuncts in recognizing variant forms. Although these proteins can be detected in other neurological diseases accompanied with substantial brain damage such as encephalitis, they are also characterized by their high sensitivity and specificity with regard to other dementing processes (Alzheimer and vascular dementia). The increase in the number of positive cases during the last years in Germany reflects an improved case ascertainment rather than the appearance of the variant CJD (vCJD). Although several recent cases with a long duration of the disease were actually recognized, they did not reveal the typical florid plaques at autopsy. They were revealed as a rare variant of sporadic CJD, which is characterized by homocygosity for valine at codon 129 and PrPSc type 1. This variant is positive for the 14-3-3 protein in CSF. Further subtypes described by Parchi et al. [5] can also be characterized by a certain pattern of clinical symptomatology, EEG- and 14-3-3-findings. In addition, differential diagnosis revealed some treatable dementias among the most common diseases (Alzheimer and vascular dementia) such as herpes encephalitis, multiple sclerosis and Hashimoto encephalitis, particularly in the younger age group.

Keywords

Vascular Dementia Prion Disease Akinetic Mutism Prpsc Type Treatable Dementia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Wien 2000

Authors and Affiliations

  • S. Poser
    • 1
  • I. Zerr
    • 1
  • A. Schroeter
    • 1
  • M. Otto
    • 1
  • A. Giese
    • 3
  • B. J. Steinhoff
    • 2
  • H. A. Kretzschmar
    • 3
  1. 1.Departments of NeurologyUniversity of GoettingenGoettingenGermany
  2. 2.Departments of NeurophysiologyUniversity of GoettingenGoettingenGermany
  3. 3.Departments of NeuropathologyUniversity of GoettingenGoettingenGermany

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