S100B and NSE serum concentrations in Machado Joseph disease.
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.
Tort
Adriano B L
AB
Departamento de Bioquímica, ICBS, UFRGS, Ramiro Barcelos 2600 anexo, CEP 90035-003, Porto Alegre, Brazil. adrianotort@hotmail.com
Portela
Luis V C
LV
Rockenbach
Isabel C
IC
Monte
Thais L
TL
Pereira
Maria L
ML
Souza
Diogo O
DO
Rieder
Carlos R M
CR
Jardim
Laura B
LB
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*
D015415:Biomarkers; D009414:Nerve Growth Factors; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins; C579160:S100B protein, human; D010751:Phosphopyruvate Hydratase