title: S100B and NSE serum concentrations in Machado Joseph disease.
abstract: BACKGROUND
  NSE and S100B are considered as neuronal and glial peripheral markers of central nervous system pathologies, respectively. We evaluated the potential use of S100B and NSE serum concentrations as peripheral markers of symptomatic patients with Machado Joseph disease (MJD).
  
  
  METHODS
  We measured S100B and NSE peripheral concentrations of 22 MJD patients and compared with healthy subjects concentrations. The correlations of both markers with CAG repeat size, age of onset, disease duration, and the scores of the Extended Disability Status Scale of Kurtzke, Unified Parkinson's Disease Rating Scale, and the Montgomery-Asberg depression rating scale were also assessed.
  
  
  RESULTS
  S100B serum concentrations between control and MJD subjects were not statistically different, whereas NSE serum concentrations were higher in MJD patients than in control subjects (p=0.00001). S100B presented a moderate correlation with disease duration and depression score, whereas NSE presented a moderate correlation with depression score and a good negative correlation with EDSS score.
  
  
  CONCLUSIONS
  Symptomatic MJD patients present increased concentrations of NSE and normal concentrations of S100B in blood.
authors.affiliation: Departamento de Bioquímica, ICBS, UFRGS, Ramiro Barcelos 2600 anexo, CEP 90035-003, Porto Alegre, Brazil. adrianotort@hotmail.com
authors.forename: Adriano B L
authors.initials: AB
authors.lastname: Tort
authors.forename: Luis V C
authors.initials: LV
authors.lastname: Portela
authors.forename: Isabel C
authors.initials: IC
authors.lastname: Rockenbach
authors.forename: Thais L
authors.initials: TL
authors.lastname: Monte
authors.forename: Maria L
authors.initials: ML
authors.lastname: Pereira
authors.forename: Diogo O
authors.initials: DO
authors.lastname: Souza
authors.forename: Carlos R M
authors.initials: CR
authors.lastname: Rieder
authors.forename: Laura B
authors.initials: LB
authors.lastname: Jardim
mesh_terms: D000328:Adult; D015415:Biomarkers; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D017827:Machado-Joseph Disease / Q000097:blood* / Q000503:physiopathology / Q000523:psychology; D008297:Male; D008875:Middle Aged; D009414:Nerve Growth Factors; D009483:Neuropsychological Tests; D010751:Phosphopyruvate Hydratase / Q000097:blood*; D020449:Repetitive Sequences, Amino Acid; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins / Q000097:blood*
chemical_list: D015415:Biomarkers; D009414:Nerve Growth Factors; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins; C579160:S100B protein, human; D010751:Phosphopyruvate Hydratase