Magnetic resonance spectroscopy and measurement of tau epitopes of autopsy proven sporadic Creutzfeldt-Jakob disease in a patient with non-specific initial EEG, MRI and negative 14-3-3 immunoblot

Šarac, Helena and Hajnšek, Sanja and Bašić, Silvio and Henigsberg, Neven and Radoš, Marko and Šimić, Goran (2008) Magnetic resonance spectroscopy and measurement of tau epitopes of autopsy proven sporadic Creutzfeldt-Jakob disease in a patient with non-specific initial EEG, MRI and negative 14-3-3 immunoblot. Collegium Antropologicum, 32 (S. 1). pp. 199-204. ISSN 0350-6134

Preview

PDF - Accepted Version Download (401kB) | Preview

Abstract

Limited potential of electroencephalogram (EEG), magnetic resonance images (MRI) and cerebrospinal fluid (CSF) test for 14-3-3 protein in the clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) resulted in developments in diagnostic premortem tehniques. Recent studies provided evidence that magnetic resonance spectroscopy (MRS) and measurement of total-tau (T-tau) and phospho-tau (P-tau) may be useful to identify patients with CJD. We combined detected metabolic changes in the brain by MRS and measured T-tau and tau-pT181 by ELISA, and tau-pT231 by Westernblot in a patient with autopsy proven sCJD. Our results show that in contrast to negative CSF 14-3-3 protein, nonspecific EEG and MRI, MRS revealed metabolic alterations in regions of the brain that has appeared normal on MRI, and tau tests has shown measurable levels of phosphorylated and non-phosphorylated isoforms in CSF. We conclude that rapidly progressive dementia with negative 14-3-3 test and non-specific initial EEG and MRI must still be considered in the differential diagnosis of the sCJD. Combination of serial functional MRI along with MRS study and measurement of tau ratio could improve the early diagnosis of sCJD. The current case is the first attempt to study results of the use of MRS and tau tests in a case of sCJD with diagnostic dilemma.

Abstract in Croatian

Ograničene mogućnosti elektroencefalografije (EEG), magnetske rezonancije (MRI) i testa na 14-3-3 protein u cerebrospinalnom likvoru (CSL) utjecale su na razvitak novih dijagnostičkih metoda u dijagnostici sporadičnog oblika Creutzfeldt-Jakobove bolesti (sCJB) premortalno. Novije studije pokazale su da spektroskopija magnetskom rezonancijom (MRS) i mjerenje koncentracije tau epitopa mogu u CSL koristiti u identificiranju oboljelih of sCJB. Mi smo simultano određivali metaboličke promjene mozga koristeći MRS i mjerili koncentracije ukupnog i fosforiliranog tau proteina (tau-pT181) u CSL koristeći ELISA metodu, te odre|ivali prisutnost tau-pT231proteina metodom Western blot u CSL pacijentice sa obdukcijom dokazanom sCJB. Naši rezultati pokazuju da usprkos negativnom nalazu 14-3-3 proteina u CSL, nespecifičnim promjenama na inicijalnom EEG-u i MRI, protonska spektroskopija je otkrila specifične metaboličke promjene i u regijama mozga koje su se doimale normalnim na MRI, a tau testovi su pokazali povišene vrijednosti nefosforiliranog i fosforiliranog tau proteina u CSL. Zaključujemo da kod brzo progresivne demencije sa negativnim 14-3-3 testom u CSL, uz nespecifične EEG i MRI promjene, ne treba isključiti sCJB. Kombinacija serijskog funkcionalnog MRI (fMRI), protonske spektroskopije i MRS uz mjerenje koncentracije nefosforiliranog i ukupnog tau proteina u CSL može unaprijediti dijagnostiku sCJB. Prema našim spoznajama, ova studija po prvi put opisuje istovremenu primjenu MRS tehnike i tau testova u identifikaciji dijagnostički dvojbenog oblika sCJB.

Actions (login required)

View Item

Downloads