Prion diseases

New considerations

Ádám Annus, Anett Csáti, L. Vécsei

Research output: Contribution to journalReview article

9 Citations (Scopus)

Abstract

The transmissible spongiform encephalopathies, which include Creutzfeldt-Jakob disease, are fatal neurodegenerative disorders caused by the pathological accumulation of abnormal prion protein. The diagnosis of Creutzfeldt-Jakob disease is complex. The electroencephalogram, magnetic resonance imaging, lumbar puncture and genetic testing findings can help in the differential diagnosis of rapidly progressive dementia. There has recently been considerable debate as to whether proteins involved in the development of neurodegenerative diseases should be regarded as prions or only share prion-like mechanisms. Two recent reports described the detection of abnormal prion protein in the nasal mucosa and urine of patients with Creutzfeldt-Jakob disease. These findings raise major health concerns regarding the transmissibility of human prion diseases. We set out to address this neurological hot topic and to draw conclusions on the basis of what is known in the literature thus far.

Original languageEnglish
Pages (from-to)125-132
Number of pages8
JournalClinical Neurology and Neurosurgery
Volume150
DOIs
Publication statusPublished - Nov 1 2016

Fingerprint

Creutzfeldt-Jakob Syndrome
Prion Diseases
Prions
Neurodegenerative Diseases
Spinal Puncture
Nasal Mucosa
Genetic Testing
Dementia
Electroencephalography
Differential Diagnosis
Magnetic Resonance Imaging
Urine
Health
Proteins
Prion Proteins

Keywords

  • Creutzfeldt-Jakob disease
  • Diagnosis
  • Neurodegenerative diseases
  • Prion protein
  • Transmissibility

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Prion diseases : New considerations. / Annus, Ádám; Csáti, Anett; Vécsei, L.

In: Clinical Neurology and Neurosurgery, Vol. 150, 01.11.2016, p. 125-132.

Research output: Contribution to journalReview article

Annus, Ádám ; Csáti, Anett ; Vécsei, L. / Prion diseases : New considerations. In: Clinical Neurology and Neurosurgery. 2016 ; Vol. 150. pp. 125-132.
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