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Short TE Quantitative Proton Magnetic Resonance Spectroscopy in Variant Creutzfeldt-Jakob Disease

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Abstract

Variant Creutzfeldt-Jakob disease (vCJD) is a fatal neurodegerative disorder. Clinical diagnosis is difficult in the early stages as the disease often presents with non-specific psychiatric and neurological symptoms. To investigate the diagnostic potential of quantitative short TE in vivo MRS, and the nature and anatomical distribution of biochemical abnormalities in vCJD, localised single-voxel spectra (TE/TR 30 ms/2,000 ms) were acquired from three brain regions: thalami, caudate nuclei and frontal white matter. Metabolite concentrations and ratios from three patients with definite or probable vCJD were compared with eight normal age-matched controls. Abnormal signal on T2-weighted MRI was apparent in the pulvinar region in all vCJD patients; this region also showed greatly increased myo-inositol [MI] (mean 2.5-fold, P=0.01) and decreased N-acetyl-aspartate (NAA; mean 2-fold, P=0.01). Two patients also showed increased [MI] (z=17, 11; one with decreased NAA, z=-12) in normal-appearing caudate nuclei. The magnitude of metabolite abnormalities in the thalami in moderately advanced vCJD suggests a potential role in earlier diagnosis. Short TE protocols allow the measurement of MI, which adds discriminant power to the MRS examination.

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References

  1. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347:921–925

    Article  PubMed  CAS  Google Scholar 

  2. Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey LJ, Lantos P (1997) The same prion strain causes vCJD and BSE. Nature 389:448–450

    Article  PubMed  CAS  Google Scholar 

  3. Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H, Bostock CJ (1997) Transmissions to mice indicate that “new variant” CJD is caused by BSE. Nature 389:498–501

    Article  PubMed  CAS  Google Scholar 

  4. Will RG, Zeidler M, Stewart GE (2000) Diagnosis of New Variant Creutzfeldt-Jakob Disease. Ann Neurol 47:575–582

    Article  PubMed  CAS  Google Scholar 

  5. UK Creutzfeldt-Jakob Disease Surveillance Unit. CJD figures. http://www.cjd.ed.ac.uk/figures.htm

  6. Andrews NJ. Quarterly Report. Incidence of variant Creutzfeldt-Jakob disease onsets and deaths in the UK, January 1994-June 2005. Statistics Unit, CDSC, Health Protection Agency. http://www.cjd.ed.ac.uk/vchdqjun05.htm

  7. Asante EA, Linehan JM, Desbruslais M, Joiner S, Gowland I, Wood AL, Welch J, Hill AF, Lloyd SE, Wadsworth JD, Collinge J (2002) BSE prions propagate as either variant CJD-like or sporadic CJD-like strains in transgenic mice expressing human prion protein. EMBO J 21(23):6358–6366

    Article  PubMed  CAS  Google Scholar 

  8. Zeidler M, Sellar RJ, Collie DA et al (2000) The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 355:1412–1418

    Article  PubMed  CAS  Google Scholar 

  9. Bahn MM, Parchi P (1999) Abnormal diffusion-weighted magnetic resonance images in Creutzfeldt-Jakob disease. Arch Neurol 56:577–583

    Article  PubMed  CAS  Google Scholar 

  10. Demaerel P, Heiner L, Robberecht W, Sciot R, Wilms G (1999). Diffusion-weighted MRI in sporadic Creutzfeldt-Jakob disease. Neurology 52:205–208

    Article  PubMed  CAS  Google Scholar 

  11. Demaerel P, Baert AL, Vanopdenbosch L, Robberecht W, Dom R (1997) Diffusion-weighted magnetic resonance imaging in Creutzfeldt-Jakob disease. Lancet 349:847–848

    Article  PubMed  CAS  Google Scholar 

  12. Waldman AD, Jarman P, Merry RTG (2003) Rapid echoplanar imaging in variant Creutzfeldt-Jakob disease: where speed is of the essence. Neuroradiology 45(8):528–531

    Article  PubMed  Google Scholar 

  13. Matoba M, Tonami H, Miyaji H, Yokota H, Yamamoto I (2001) Creutzfeldt-Jakob disease: serial changes on diffusion-weighted MRI. J Comput Assist Tomogr 25(2):274–277

    Article  PubMed  CAS  Google Scholar 

  14. Bruhn H, Weber T, Thorwirth V, Frahm J (1991) In-vivo monitoring of neuronal loss in Creutzfeldt-Jakob disease by proton magnetic resonance spectroscopy. Lancet 337:1610–1611

    Article  PubMed  CAS  Google Scholar 

  15. Graham GD, Petroff OAC, Blamire AM, Rajkowska G, Goldman-Rakic P, Prichard JW (1993) Proton magnetic resonance spectroscopy in Creutzfeldt-Jakob disease. Neurology 43:2065–2068

    PubMed  CAS  Google Scholar 

  16. Shyu W-C, Lee C-C, Hsu Y-D et al (1996) Panencephalitic Creutzfeldt-Jakob disease. Unusual presentation of magnetic resonance spectroscopy. J Neurol Sci 138:157–160

    Article  PubMed  CAS  Google Scholar 

  17. Konaka K, Kaido M, Okuda Y et al (2000) Proton magnetic resonance spectroscopy of a patient with Gerstmann-Straussler-Scheinker disease. Neuroradiology 42:662–665

    Article  PubMed  CAS  Google Scholar 

  18. Galanaud D, Dormont D, Grabli D, Charles P, Hauw JJ, Lubetzki C, Brandel JP, Marsault C, Cozzone PJ (2002) MR spectroscopic pulvinar sign in a case of variant Creutzfeldt-Jakob disease. J Neuroradiol 29(4):285–287

    PubMed  CAS  Google Scholar 

  19. Pandya HG, Coley SC, Wilkinson ID and Griffiths PD (2003) Magnetic resonance spectroscopy abnormalities in sporadic and variant Creutzfeldt-Jakob disease. Clin Radiol 58:148–153

    Article  PubMed  CAS  Google Scholar 

  20. Provencher SW (1993) Estimation of metabolite concentrations from localized in vivo proton NMR spectra. Magn Reson Med 30:672–679

    Article  PubMed  CAS  Google Scholar 

  21. Valenzuela MJ, Sachdev P (2001). Magnetic resonance spectroscopy in AD. Neurology 56:592–598

    PubMed  CAS  Google Scholar 

  22. Behar KL, Boucher R, Fritch W, Manuelidis L (1998) Changes in N-acetylaspartate and myo-inositol detected in the cerebral cortex of hamsters with Creutzfeldt-Jakob disease. Magn Reson Imaging 16:963–968

    Article  PubMed  CAS  Google Scholar 

  23. Cheng LL, Newell K, Mallory AE, Hyman BT, Gonzalez RG (2002) Quantification of neurons in Alzheimer and control brains with ex vivo high-resolution magic angle spinning proton magnetic resonance spectroscopy and stereology. Magn Reson Imaging 20(7):527–533

    Article  PubMed  Google Scholar 

  24. Salibi N, Brown MA (1997) Clinical MR spectroscopy. First principles. Chapter 6; 143–202. Wiley-Liss, John Wiley & Sons, Inc. USA

    Google Scholar 

  25. Brand A, Richter-Landsberg C, Leibfritz D (1993) Multinuclear NMR studies on the energy metabolism of glial and neuronal cells. Dev Neurosci 15(3–5):289–298

    Article  PubMed  CAS  Google Scholar 

  26. Strange K, Emma F, Paredes A, Morrison R (1994) Osmoregulatory changes in myo-inositol content and Na+/myo-inositol cotransport in rat cortical astrocytes. Glia 12(1):35–43

    Article  PubMed  CAS  Google Scholar 

  27. Ironside JW, Bell JE (1997) Florid plaques and new variant Creutzfeldt-Jakob disease. Lancet 350:1475

    Article  PubMed  CAS  Google Scholar 

  28. Huang W, Alexander GE, Daly EM, Shetty HU, Krasuski JS, Rapoport SI, Schapiro MB (1996) High brain myo-inositol levels in the predementia phase of Alzheimer’s disease in adults with Down’s syndrome. Am J Psychiatry 156:1879–1886

    Google Scholar 

  29. Kantarci K, Jack CR, Xu YC, Campeau NG, O’Brien PC, Smith GE, Ivnik RJ, Boeve BF, Kokmen E, Tangalos EG, Petersen RC (2000) Regional metabolic patterns in mild cognitive impairment and Alzheimer’s disease. Neurology 55:210–217

    PubMed  CAS  Google Scholar 

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Acknowledgements

The authors would like to acknowledge the assistance of Hilary Watt, statistician at London School for Hygiene and Tropical Medicine, Professor John Collinge in the Prion Unit, University College London, and their colleagues in the Dementia Research Group. This work was supported by the Medical Research Council of Great Britain.

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Correspondence to A. D. Waldman.

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Cordery, R.J., MacManus, D., Godbolt, A. et al. Short TE Quantitative Proton Magnetic Resonance Spectroscopy in Variant Creutzfeldt-Jakob Disease. Eur Radiol 16, 1692–1698 (2006). https://doi.org/10.1007/s00330-005-0090-4

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  • DOI: https://doi.org/10.1007/s00330-005-0090-4

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