Neuroradiology

, Volume 52, Issue 12, pp 1079–1085 | Cite as

Proton magnetic resonance spectroscopy in patients with early stages of amyotrophic lateral sclerosis

  • Štefan Sivák
  • Michal Bittšanský
  • Egon Kurča
  • Monika Turčanová-Koprušáková
  • Milan Grofik
  • Vladimír Nosáľ
  • Hubert Poláček
  • Dušan Dobrota
Diagnostic Neuroradiology

Abstract

Introduction

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder primarily affecting upper and lower motor neurons. Due to relative fast progression of the disease, early diagnosis is essential. Proton magnetic resonance spectroscopy (1H-MRS) is used for objectivization of upper motor neuron (UMN) lesions. The aim of this study was to assess the use of 1H-MRS in the early stages of ALS.

Methods

Eleven patients with clinically definite (n = 2), probable (n = 7), and probable laboratory-supported (n = 2) diagnosis of ALS with disease duration of less than 14 months were studied. Control group consists of 11 sex- and age-matched healthy subjects. All subjects underwent assessment of functional disability using revised ALS Functional Rating Scale (ALSFRS-R) and single-voxel 1H-MRS examination of both precentral gyri, pons, medulla oblongata, and occipital lobe. Spectra were evaluated with LCModel software.

Results

The mean disease duration was 6.5 ± 3.5 months. The median ALSFRS-R was 42. Significant decrease between patient and control groups was found in the NAA/Cre ratio in the left and right precentral gyri (p = 0.008, p = 0.040). Other metabolite ratios in other areas did not show significant differences. Total ALSFRS-R score weakly positively correlated with NAA/Cre ratio in the left precentral gyrus (p = 0.047).

Conclusions

1H-MRS is sensitive to detect metabolic changes caused by neurodegeneration processes during ALS and can be used for detection of UMN dysfunction. These MRS changes in the early stages of ALS are most prominent in motor cortex.

Keywords

Amyotrophic lateral sclerosis Magnetic resonance spectroscopy Clinical findings ALSFRS-R 

References

  1. 1.
    Logroscino G, Traynor BJ, Hardiman O, Chio' A, Couratier P, Mitchell JD, Swingler RJ, Beghi E (2008) EURALS. Descriptive epidemiology of amyotrophic lateral sclerosis: new evidence and unsolved issues. J Neurol Neurosurg Psychiatry 79(1):6–11CrossRefPubMedGoogle Scholar
  2. 2.
    National Health Information Center of Slovak Republic (2009) http://www.nczisk.sk. Accessed 2 Sep 2009
  3. 3.
    Wijesekera LC, Leigh PN (2009) Amyotrophic lateral sclerosis. Orphanet J Rare Dis 4:3CrossRefPubMedGoogle Scholar
  4. 4.
    Turner MR, Kiernan MC, Leigh PN, Talbot K (2009) Biomarkers in amyotrophic lateral sclerosis. Lancet Neurol 8(1):94–109CrossRefPubMedGoogle Scholar
  5. 5.
    Mandrioli J, Faglioni P, Nichelli P, Sola P (2006) Amyotrophic lateral sclerosis: prognostic indicators of survival. Amyotroph Lateral Scler 7(4):211–220CrossRefPubMedGoogle Scholar
  6. 6.
    Andersen PM, Borasio GD, Dengler R, Hardiman O, Kollewe K, Leigh PN, Pradat PF, Silani V, Tomik B (2006) Amyotrophic lateral sclerosis. In: Hughes R, Brainin R et al (eds) European Handbook of Neurological Management. Blackwell, Oxford, pp 299–321CrossRefGoogle Scholar
  7. 7.
    Chiò A (1999) ISIS survey: an international study on the diagnostic process and its implications in amyotrophic lateral sclerosis. J Neurol 246(Suppl 3):1–5CrossRefGoogle Scholar
  8. 8.
    Brooks BR, Miller RG, Swash M, Munsat TL (2000) World federation of neurology research group on motor neuron diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Mot Neuron Disord 1(5):293–299CrossRefGoogle Scholar
  9. 9.
    Murray B, Mitsumoto H (2008) Disorders of upper and lower motor neurons. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J (eds) Neurology in clinical practice, 5th edn. Butterworth Heinemann Elsevier, Philadelphia, pp 2203–2212Google Scholar
  10. 10.
    Provencher SW (1993) Estimation of metabolite concentrations from localized in vivo proton NMR spectra. Magn Reson Med 30(6):672–679CrossRefPubMedGoogle Scholar
  11. 11.
    Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169(1–2):13–21CrossRefPubMedGoogle Scholar
  12. 12.
    Chan S, Shungu DC, Douglas-Akinwande A, Lange DJ, Rowland LP (1999) Motor neuron diseases: comparison of single-voxel proton MR spectroscopy of the motor cortex with MR imaging of the brain. Radiology 212(3):763–769PubMedGoogle Scholar
  13. 13.
    Gredal O, Rosenbaum S, Topp S, Karlsborg M, Strange P, Werdelin L (1997) Quantification of brain metabolites in amyotrophic lateral sclerosis by localized proton magnetic resonance spectroscopy. Neurology 48(4):878–881PubMedGoogle Scholar
  14. 14.
    Block W, Karitzky J, Träber F, Pohl Ch, Keller E, Mundegar RR, Lamerichs R, Rink H, Ries F, Schild HH, Jerusalem F (1998) Proton magnetic resonance spectroscopy of the primary motor cortex in patients with motor neuron disease. Arch Neurol 55:931–936CrossRefPubMedGoogle Scholar
  15. 15.
    Pohl C, Block W, Karitzky J, Träber F, Schmidt S, Grothe C, Lamerichs R, Schild H, Klockgether T (2001) Proton magnetic resonance spectroscopy of the motor cortex in 70 patients with amyotrophic lateral sclerosis. Arch Neurol 58(5):729–735CrossRefPubMedGoogle Scholar
  16. 16.
    Suhy J, Miller RG, Rule R, Schuff N, Licht J, Dronsky V, Gelinas D, Maudsley AA, Weiner MW (2002) Early detection and longitudinal changes in amyotrophic lateral sclerosis by (1)H MRSI. Neurology 58(5):773–779PubMedGoogle Scholar
  17. 17.
    Rule RR, Suhy J, Schuff N, Gelinas DF, Miller RG, Weiner MW (2004) Reduced NAA in motor and non-motor brain regions in amyotrophic lateral sclerosis: a cross-sectional and longitudinal study. Amyotroph Lateral Scler Other Mot Neuron Disord 5(3):141–149CrossRefGoogle Scholar
  18. 18.
    Pioro EP, Antel JP, Cashman NR, Arnold DL (1994) Detection of cortical neuron loss in motor neuron disease by proton magnetic resonance spectroscopic imaging in vivo. Neurology 44(10):1933–1938PubMedGoogle Scholar
  19. 19.
    Bowen BC, Pattany PM, Bradley WG, Murdoch JB, Rotta F, Younis AA, Duncan RC, Quencer RM (2000) MR imaging and localized proton spectroscopy of the precentral gyrus in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol 21(4):647–658PubMedGoogle Scholar
  20. 20.
    Unrath A, Ludolph AC, Kassubek J (2007) Brain metabolites in definite amyotrophic lateral sclerosis. A longitudinal proton magnetic resonance spectroscopy study. J Neurol 254(8):1099–1106CrossRefPubMedGoogle Scholar
  21. 21.
    Sarchielli P, Pelliccioli GP, Tarducci R, Chiarini P, Presciutti O, Gobbi G, Gallai V (2001) Magnetic resonance imaging and 1H-magnetic resonance spectroscopy in amyotrophic lateral sclerosis. Neuroradiology 43(3):189–197CrossRefPubMedGoogle Scholar
  22. 22.
    Tarducci R, Sarchielli P, Presciutti O, Pelliccioli GP, Gentile E, Gobbi G (2000) Study of the primary motor cortex in amyotrophic lateral sclerosis by quantitative 1HMRSI. ISMRM 1:632Google Scholar
  23. 23.
    Bradley WG, Bowen BC, Pattany PM, Rotta F (1999) 1H-magnetic resonance spectroscopy in amyotrophic lateral sclerosis. J Neurol Sci 169(1–2):84–86CrossRefPubMedGoogle Scholar
  24. 24.
    Giroud M, Walker P, Bernard D, Lemesle M, Martin D, Baudouin N, Brunotte F, Dumas R (1996) Reduced brain N-acetyl-aspartate in frontal lobes suggests neuronal loss in patients with amyotrophic lateral sclerosis. Neurol Res 18(3):241–243PubMedGoogle Scholar
  25. 25.
    Vielhaber S, Kaufmann J, Kanowski M, Sailer M, Feistner H, Tempelmann C, Elger CE, Heinze HJ, Kunz WS (2001) Effect of creatine supplementation on metabolite levels in ALS motor cortices. Exp Neurol 172(2):377–382CrossRefPubMedGoogle Scholar
  26. 26.
    Wang S, Poptani H, Woo JH, Desiderio LM, Elman LB, McCluskey LF, Krejza J, Melhem ER (2006) Amyotrophic lateral sclerosis: diffusion-tensor and chemical shift MR imaging at 3.0 T. Radiology 239(3):831–838CrossRefPubMedGoogle Scholar
  27. 27.
    Strong MJ, Grace GM, Orange JB, Leeper HA, Menon RS, Aere C (1999) A prospective study of cognitive impairment in ALS. Neurology 53(8):1665–1670PubMedGoogle Scholar
  28. 28.
    Kalra S, Cashman NR, Caramanos Z, Genge A, Arnold DL (2003) Gabapentin therapy for amyotrophic lateral sclerosis: lack of improvement in neuronal integrity shown by MR spectroscopy. AJNR Am J Neuroradiol 24(3):476–480PubMedGoogle Scholar
  29. 29.
    Kalra S, Vitale A, Cashman NR, Genge A, Arnold DL (2006) Cerebral degeneration predicts survival in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 77(11):1253–1255CrossRefPubMedGoogle Scholar
  30. 30.
    Abe K, Takanashi M, Watanabe Y, Tanaka H, Fujita N, Hirabuki N, Yanagihara T (2001) Decrease in N-acetyl aspartate/creatine ratio in the motor area and the frontal lobe in amyotrophic lateral sclerosis. Neuroradiology 43(7):537–541CrossRefPubMedGoogle Scholar
  31. 31.
    Wang S, Melhem ER (2005) Amyotrophic lateral sclerosis and primary lateral sclerosis: the role of diffusion tensor imaging and other advanced MR-based techniques as objective upper motor neuron markers. Ann N Y Acad Sci 1064:61–77CrossRefPubMedGoogle Scholar
  32. 32.
    Ellis CM, Simmons A, Andrews C, Dawson JM, Williams SC, Leigh PN (1998) A proton magnetic resonance spectroscopic study in ALS: correlation with clinical findings. Neurology 51(4):1104–1109PubMedGoogle Scholar
  33. 33.
    Pioro EP, Majors AW, Mitsumoto H, Nelson DR, Ng TC (1999) 1H-MRS evidence of neurodegeneration and excess glutamate + glutamine in ALS medulla. Neurology 53(1):71–79PubMedGoogle Scholar
  34. 34.
    Schuff N, Rooney WD, Miller R, Gelinas DF, Amend DL, Maudsley AA, Weiner MW (2001) Reanalysis of multislice (1)H MRSI in amyotrophic lateral sclerosis. Magn Reson Med 45(3):513–516CrossRefPubMedGoogle Scholar
  35. 35.
    Rooney WD, Miller RG, Gelinas D, Schuff N, Maudsley AA, Weiner MW (1998) Decreased N-acetyl aspartate in motor cortex and corticospinal tract in ALS. Neurology 50(6):1800–1805PubMedGoogle Scholar
  36. 36.
    Cwik VA, Hanstock CC, Allen PS, Martin WR (1998) Estimation of brainstem neuronal loss in amyotrophic lateral sclerosis with in vivo proton magnetic resonance spectroscopy. Neurology 50(1):72–77PubMedGoogle Scholar
  37. 37.
    Han J, Ma L (2010) Study of the features of proton MR spectroscopy ((1)H-MRS) on amyotrophic lateral sclerosis. J Magn Reson Imaging 31(2):305–308CrossRefPubMedGoogle Scholar
  38. 38.
    Barker PB, Lin DDM (2006) In vivo proton MR spectroscopy of the human brain. Prog Nucl Magn Reson Spectrosc 49:99–128CrossRefGoogle Scholar
  39. 39.
    Kaiser LG, Schuff N, Cashdollar N, Weiner MW (2005) Age-related glutamate and glutamine concentration changes in normal human brain: 1H MR spectroscopy study at 4 T. Neurobiol Aging 26(5):665–672CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Štefan Sivák
    • 1
    • 4
  • Michal Bittšanský
    • 2
  • Egon Kurča
    • 1
  • Monika Turčanová-Koprušáková
    • 1
  • Milan Grofik
    • 1
  • Vladimír Nosáľ
    • 1
  • Hubert Poláček
    • 3
  • Dušan Dobrota
    • 2
  1. 1.Clinic of Neurology, Jessenius Faculty of MedicineComenius UniversityMartinSlovak Republic
  2. 2.Department of Medical Biochemistry, Jessenius Faculty of MedicineComenius UniversityMartinSlovak Republic
  3. 3.Clinic of Radiodiagnostics, Jessenius Faculty of MedicineComenius UniversityMartinSlovak Republic
  4. 4.Jessenius Medical Faculty, Clinic of NeurologyMartinSlovakia

Personalised recommendations