NR AWFR

AU Gmitterova,K.; Karsnianski,A.; Heinemann,U.; Bodemer,M.; Ciesielczyk,B.; Zerr,I.

TI Cerebrospinal fluid profile in Huntingdon disease

QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions DIA-18

PT Konferenz-Poster

AB Cerebrospinal fluid (CSF) biomarkers in patients with Huntington disease (HD), Creutzfeldt-Jakob disease (CJD), normal pressure hydrocephalus (NPH), and in healthy controls were evaluated for their potential diagnostic value. Cerebrospinal fluid was assayed for tau, amyloid ß 1-42 (Aß 1-42), neuron-specific enolase (NSE), astrocytic protein S 100b, and cellular prion protein (PrPc) using commercially available ELISA. Tests were conducted in 15 patients with HD, 17 definite CJD cases with negative 14-3-3 protein (CJD-), 19 definite CJD cases with 14-3-3 positive (CJD+), and in 12 healthy controls. The CSF levels of tau and Aß 1-42 were significantly different in HD compared with CJD- (p=0.007 and p=<0.001). There were significant differences of tau (p=<0.001), NSE (p=<0.001) and S 100b (p=<0.001) levels between CJD+ and HD group. No significant difference between HD and healthy controls was found. A significant elevation of NSE level in HD group compared with NPH was detected, as well. In addition, the 14-3-3 positive CJD group exhibited significantly elevated tau (p=<0.001), Aß 1-42 (p=0.029), NSE (p=<0.001) and S 100b (p=0.001) levels in comparison with 14-3-3 negative CJD group. No significant differences of PrPc levels between HD and other groups neither differences between CJD+ and CJD- groups were found. No difference of tau, Aß 1-42, NSE, S 100b levels between patients with HD and healthy controls was found. Long disease duration, different pathophysiological processes in demential disorders and specific lesion profile in HD may be possible causes of these findings. The mechanism of 14-3-3 elevation and release of other markers into CSF in different stages of CJD is still unknown. However, there is a correlation of CSF marker levels with 14-3-3 positivity/negativity.

AD Department of Neurology, Georg-August University Göttingen, Germany. E-mail: epicjd@med.uni-goettingen.de

SP englisch

PO Italien

EA Poster

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