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Alzheimers Dement. 2017 Jun;13(6):710-719. doi: 10.1016/j.jalz.2016.09.013. Epub 2016 Nov 18.

Evaluation of α-synuclein as a novel cerebrospinal fluid biomarker in different forms of prion diseases.

Author information

1
Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany. Electronic address: franc.llorens@gmail.com.
2
Institute for Neuropathology, University Medical Center Göttingen, Göttingen, Germany.
3
Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany.
4
Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland.
5
Laboratory of Pharmacology, School of Health Sciences, Department of Pharmacy, Aristotle University of Thessaloniki, Thessaloniki, Greece.
6
Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany.
7
Department of Prion Diseases, Slovak Medical University Bratislava, Bratislava, Slovakia.
8
Institute for Neuropathology, University Medical Center Göttingen, Göttingen, Germany; Paracelsus-Elena Klinik, Center for Parkinsonism and Movement Disorders, Kassel, Germany; Department of Neurosurgery, University Medical Center Göttingen, Göttingen, Germany.

Abstract

INTRODUCTION:

Accurate diagnosis of prion diseases and discrimination from alternative dementias gain importance in the clinical routine, but partial overlap in cerebrospinal fluid (CSF) biomarkers impedes absolute discrimination in the differential diagnostic context.

METHODS:

We established the clinical parameters for prion disease diagnosis for the quantification of CSF α-synuclein in patients with sporadic (n = 234) and genetic (n = 56) prion diseases, in cases with cognitive impairment/dementia or neurodegenerative disease (n = 278), and in the neurologic control group (n = 111).

RESULTS:

An optimal cutoff value of 680 pg/mL α-synuclein results in 94% sensitivity and 96% specificity when diagnosing sporadic Creutzfeldt-Jakob disease (CJD). Genetic CJD cases showed increased CSF α-synuclein values. No increased α-synuclein levels were detected in non-CJD cases with rapid progression course.

DISCUSSION:

Detection of α-synuclein in the CSF of patients with suspected CJD is a valuable diagnostic test reaching almost full discrimination from non-prion disease cases. These data highlight the utility of CSF α-synuclein quantification in front of classical CSF biomarkers in clinical routine.

KEYWORDS:

Biomarker; Cerebrospinal fluid; ELISA; Genetic Creutzfeldt-Jakob disease; Neurodegenerative diseases; Prion diseases; Sporadic Creutzfeldt-Jakob disease; α-Synuclein

PMID:
27870938
DOI:
10.1016/j.jalz.2016.09.013
[Indexed for MEDLINE]

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