Accuracy of diagnostic criteria for sporadic Creutzfeldt-Jakob disease among rapidly progressive dementia

Matteo Tagliapietra, Gianluigi Zanusso, Michele Fiorini, Nicola Bonetto, Giulia Zarantonello, Alberto Zambon, Mario Ermani, Salvatore Monaco, Renzo Manara, Annachiara Cagnin

Research output: Contribution to journalArticle

14 Citations (Scopus)

Abstract

Rapidly progressive dementia (RPD) is a rare presentation of different neurological disorders characterized by cognitive impairment leading to loss of functional independence within 24 months or less. The increasing recognition of treatable non-prion causes of RPD has made the differential diagnosis with sporadic Creutzfeldt-Jakob disease (sCJD) of crucial importance. We therefore assessed the frequency of different etiologies of RPD and evaluated the accuracy of newly proposed diagnostic criteria for sCJD. Clinical records of patients with RPD referred to Memory Clinic between 2007 and 2012 were retrospectively analyzed. The accuracy of diagnostic criteria for sCJD was evaluated by: a) MRI images in DWI and FLAIR sequences; and (b) CSF 14-3-3 protein. In addition, CSF total tau protein level was also assessed. Final diagnosis was obtained after a 1-year follow-up or after autopsy. Among 37 patients with RPD, the most frequent causes were non-prion diseases, either untreatable (38%) or potentially treatable (32%), thus leaving sCJD as a less frequent cause (30%). DWI images had a sensitivity of 73% and specificity of 96%, while FLAIR yielded a very low sensitivity (40%). CSF 14-3-3 protein had a sensitivity of 100%, but a very low specificity (43%). The strongest independent predictor of sCJD diagnosis was the CSF tau level (p = 0.002) (91% sensitivity, 83% specificity). Treatable causes of RPD are as frequent as sCJD and a rapid differential diagnosis is mandatory. We suggest that DWI images and CSF analysis combining 14-3-3 and total tau protein determination hold the best informative diagnostic values.

Original languageEnglish
Pages (from-to)231-238
Number of pages8
JournalJournal of Alzheimer's Disease
Volume34
Issue number1
DOIs
Publication statusPublished - 2013

Fingerprint

Dementia
14-3-3 Proteins
Differential Diagnosis
Sensitivity and Specificity
tau Proteins
Nervous System Diseases
Sporadic Creutzfeldt-Jakob Disease
Autopsy

Keywords

  • Creutzfeldt-Jakob disease
  • limbic encephalitis
  • rapidly progressive dementia
  • tau protein

ASJC Scopus subject areas

  • Psychiatry and Mental health
  • Geriatrics and Gerontology
  • Clinical Psychology

Cite this

Tagliapietra, M., Zanusso, G., Fiorini, M., Bonetto, N., Zarantonello, G., Zambon, A., ... Cagnin, A. (2013). Accuracy of diagnostic criteria for sporadic Creutzfeldt-Jakob disease among rapidly progressive dementia. Journal of Alzheimer's Disease, 34(1), 231-238. https://doi.org/10.3233/JAD-121873

Accuracy of diagnostic criteria for sporadic Creutzfeldt-Jakob disease among rapidly progressive dementia. / Tagliapietra, Matteo; Zanusso, Gianluigi; Fiorini, Michele; Bonetto, Nicola; Zarantonello, Giulia; Zambon, Alberto; Ermani, Mario; Monaco, Salvatore; Manara, Renzo; Cagnin, Annachiara.

In: Journal of Alzheimer's Disease, Vol. 34, No. 1, 2013, p. 231-238.

Research output: Contribution to journalArticle

Tagliapietra, M, Zanusso, G, Fiorini, M, Bonetto, N, Zarantonello, G, Zambon, A, Ermani, M, Monaco, S, Manara, R & Cagnin, A 2013, 'Accuracy of diagnostic criteria for sporadic Creutzfeldt-Jakob disease among rapidly progressive dementia', Journal of Alzheimer's Disease, vol. 34, no. 1, pp. 231-238. https://doi.org/10.3233/JAD-121873
Tagliapietra, Matteo ; Zanusso, Gianluigi ; Fiorini, Michele ; Bonetto, Nicola ; Zarantonello, Giulia ; Zambon, Alberto ; Ermani, Mario ; Monaco, Salvatore ; Manara, Renzo ; Cagnin, Annachiara. / Accuracy of diagnostic criteria for sporadic Creutzfeldt-Jakob disease among rapidly progressive dementia. In: Journal of Alzheimer's Disease. 2013 ; Vol. 34, No. 1. pp. 231-238.
@article{7e3b91ecafba46c384831bd6873dfdcb,
title = "Accuracy of diagnostic criteria for sporadic Creutzfeldt-Jakob disease among rapidly progressive dementia",
abstract = "Rapidly progressive dementia (RPD) is a rare presentation of different neurological disorders characterized by cognitive impairment leading to loss of functional independence within 24 months or less. The increasing recognition of treatable non-prion causes of RPD has made the differential diagnosis with sporadic Creutzfeldt-Jakob disease (sCJD) of crucial importance. We therefore assessed the frequency of different etiologies of RPD and evaluated the accuracy of newly proposed diagnostic criteria for sCJD. Clinical records of patients with RPD referred to Memory Clinic between 2007 and 2012 were retrospectively analyzed. The accuracy of diagnostic criteria for sCJD was evaluated by: a) MRI images in DWI and FLAIR sequences; and (b) CSF 14-3-3 protein. In addition, CSF total tau protein level was also assessed. Final diagnosis was obtained after a 1-year follow-up or after autopsy. Among 37 patients with RPD, the most frequent causes were non-prion diseases, either untreatable (38{\%}) or potentially treatable (32{\%}), thus leaving sCJD as a less frequent cause (30{\%}). DWI images had a sensitivity of 73{\%} and specificity of 96{\%}, while FLAIR yielded a very low sensitivity (40{\%}). CSF 14-3-3 protein had a sensitivity of 100{\%}, but a very low specificity (43{\%}). The strongest independent predictor of sCJD diagnosis was the CSF tau level (p = 0.002) (91{\%} sensitivity, 83{\%} specificity). Treatable causes of RPD are as frequent as sCJD and a rapid differential diagnosis is mandatory. We suggest that DWI images and CSF analysis combining 14-3-3 and total tau protein determination hold the best informative diagnostic values.",
keywords = "Creutzfeldt-Jakob disease, limbic encephalitis, rapidly progressive dementia, tau protein",
author = "Matteo Tagliapietra and Gianluigi Zanusso and Michele Fiorini and Nicola Bonetto and Giulia Zarantonello and Alberto Zambon and Mario Ermani and Salvatore Monaco and Renzo Manara and Annachiara Cagnin",
year = "2013",
doi = "10.3233/JAD-121873",
language = "English",
volume = "34",
pages = "231--238",
journal = "Journal of Alzheimer's Disease",
issn = "1387-2877",
publisher = "IOS Press",
number = "1",

}

TY - JOUR

T1 - Accuracy of diagnostic criteria for sporadic Creutzfeldt-Jakob disease among rapidly progressive dementia

AU - Tagliapietra, Matteo

AU - Zanusso, Gianluigi

AU - Fiorini, Michele

AU - Bonetto, Nicola

AU - Zarantonello, Giulia

AU - Zambon, Alberto

AU - Ermani, Mario

AU - Monaco, Salvatore

AU - Manara, Renzo

AU - Cagnin, Annachiara

PY - 2013

Y1 - 2013

N2 - Rapidly progressive dementia (RPD) is a rare presentation of different neurological disorders characterized by cognitive impairment leading to loss of functional independence within 24 months or less. The increasing recognition of treatable non-prion causes of RPD has made the differential diagnosis with sporadic Creutzfeldt-Jakob disease (sCJD) of crucial importance. We therefore assessed the frequency of different etiologies of RPD and evaluated the accuracy of newly proposed diagnostic criteria for sCJD. Clinical records of patients with RPD referred to Memory Clinic between 2007 and 2012 were retrospectively analyzed. The accuracy of diagnostic criteria for sCJD was evaluated by: a) MRI images in DWI and FLAIR sequences; and (b) CSF 14-3-3 protein. In addition, CSF total tau protein level was also assessed. Final diagnosis was obtained after a 1-year follow-up or after autopsy. Among 37 patients with RPD, the most frequent causes were non-prion diseases, either untreatable (38%) or potentially treatable (32%), thus leaving sCJD as a less frequent cause (30%). DWI images had a sensitivity of 73% and specificity of 96%, while FLAIR yielded a very low sensitivity (40%). CSF 14-3-3 protein had a sensitivity of 100%, but a very low specificity (43%). The strongest independent predictor of sCJD diagnosis was the CSF tau level (p = 0.002) (91% sensitivity, 83% specificity). Treatable causes of RPD are as frequent as sCJD and a rapid differential diagnosis is mandatory. We suggest that DWI images and CSF analysis combining 14-3-3 and total tau protein determination hold the best informative diagnostic values.

AB - Rapidly progressive dementia (RPD) is a rare presentation of different neurological disorders characterized by cognitive impairment leading to loss of functional independence within 24 months or less. The increasing recognition of treatable non-prion causes of RPD has made the differential diagnosis with sporadic Creutzfeldt-Jakob disease (sCJD) of crucial importance. We therefore assessed the frequency of different etiologies of RPD and evaluated the accuracy of newly proposed diagnostic criteria for sCJD. Clinical records of patients with RPD referred to Memory Clinic between 2007 and 2012 were retrospectively analyzed. The accuracy of diagnostic criteria for sCJD was evaluated by: a) MRI images in DWI and FLAIR sequences; and (b) CSF 14-3-3 protein. In addition, CSF total tau protein level was also assessed. Final diagnosis was obtained after a 1-year follow-up or after autopsy. Among 37 patients with RPD, the most frequent causes were non-prion diseases, either untreatable (38%) or potentially treatable (32%), thus leaving sCJD as a less frequent cause (30%). DWI images had a sensitivity of 73% and specificity of 96%, while FLAIR yielded a very low sensitivity (40%). CSF 14-3-3 protein had a sensitivity of 100%, but a very low specificity (43%). The strongest independent predictor of sCJD diagnosis was the CSF tau level (p = 0.002) (91% sensitivity, 83% specificity). Treatable causes of RPD are as frequent as sCJD and a rapid differential diagnosis is mandatory. We suggest that DWI images and CSF analysis combining 14-3-3 and total tau protein determination hold the best informative diagnostic values.

KW - Creutzfeldt-Jakob disease

KW - limbic encephalitis

KW - rapidly progressive dementia

KW - tau protein

UR - http://www.scopus.com/inward/record.url?scp=84874325069&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84874325069&partnerID=8YFLogxK

U2 - 10.3233/JAD-121873

DO - 10.3233/JAD-121873

M3 - Article

VL - 34

SP - 231

EP - 238

JO - Journal of Alzheimer's Disease

JF - Journal of Alzheimer's Disease

SN - 1387-2877

IS - 1

ER -