title: Evaluation of α-synuclein as a novel cerebrospinal fluid biomarker in different forms of prion diseases.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: Franc
authors.initials: F
authors.lastname: Llorens
authors.affiliation: Institute for Neuropathology, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Niels
authors.initials: N
authors.lastname: Kruse
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Schmitz
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany.
authors.forename: Nadine
authors.initials: N
authors.lastname: Gotzmann
authors.affiliation: Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland.
authors.forename: Ewa
authors.initials: E
authors.lastname: Golanska
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany.
authors.forename: Katrin
authors.initials: K
authors.lastname: Thüne
authors.affiliation: Laboratory of Pharmacology, School of Health Sciences, Department of Pharmacy, Aristotle University of Thessaloniki, Thessaloniki, Greece.
authors.forename: Orgeta
authors.initials: O
authors.lastname: Zejneli
authors.affiliation: Laboratory of Pharmacology, School of Health Sciences, Department of Pharmacy, Aristotle University of Thessaloniki, Thessaloniki, Greece.
authors.forename: Eirini
authors.initials: E
authors.lastname: Kanata
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Tobias
authors.initials: T
authors.lastname: Knipper
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany.
authors.forename: Maria
authors.initials: M
authors.lastname: Cramm
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Lange
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany.
authors.forename: Saima
authors.initials: S
authors.lastname: Zafar
authors.affiliation: Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland.
authors.forename: Beata
authors.initials: B
authors.lastname: Sikorska
authors.affiliation: Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland.
authors.forename: Pawel P
authors.initials: PP
authors.lastname: Liberski
authors.affiliation: Department of Prion Diseases, Slovak Medical University Bratislava, Bratislava, Slovakia.
authors.forename: Eva
authors.initials: E
authors.lastname: Mitrova
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Daniela
authors.initials: D
authors.lastname: Varges
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Schmidt
authors.affiliation: Laboratory of Pharmacology, School of Health Sciences, Department of Pharmacy, Aristotle University of Thessaloniki, Thessaloniki, Greece.
authors.forename: Theodoros
authors.initials: T
authors.lastname: Sklaviadis
authors.affiliation: 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.
authors.forename: Brit
authors.initials: B
authors.lastname: Mollenhauer
authors.affiliation: Department of Neurology, Clinical Dementia Center, University Medical Center Göttingen, Göttingen, Germany; German Center for Neurodegenerative Diseases (DZNE), Site Göttingen, Germany.
authors.forename: Inga
authors.initials: I
authors.lastname: Zerr
mesh_terms: D000368:Aged; D015415:Biomarkers / Q000134:cerebrospinal fluid; D015331:Cohort Studies; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D006801:Humans; D008875:Middle Aged; D017096:Prion Diseases / Q000134:cerebrospinal fluid*; D012680:Sensitivity and Specificity; D051844:alpha-Synuclein / Q000134:cerebrospinal fluid*
chemical_list: D015415:Biomarkers; C497604:SNCA protein, human; D051844:alpha-Synuclein
keywords: Biomarker; Cerebrospinal fluid; ELISA; Genetic Creutzfeldt-Jakob disease; Neurodegenerative diseases; Prion diseases; Sporadic Creutzfeldt-Jakob disease; α-Synuclein