Signs of MRI affection of brain in сlassical Amyotrophic Lateral Sclerosis patients

Cover Page
Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription Access


T2 hyperintensive focal lesions were found on brain MRI only in 41 patients (9.7 %) from the ALS group (n = 423). Female were 20 (48,8 %) and male - 21 (51,2 %), aged 31 to 78 years (middle age is 56 ± 4). Focal MRI lesions were found exclusively in projection of the intracranial section of the corticospinal tract. They were always bilateral and symmetrical and were located at one or two levels, most often in the posterior limb of internal capsule and in cerebral peduncle (85 % and 60 % correspondingly). They were less frequent in the radiate crown, semioval center or medulla. There was no reliable correlation between MRI changes and clinical type of ALS, age of disease onset, duration of symptoms and region of onset of muscle weakness and atrophy. Foci of hyperintensive MRI signal in the ALS did not change in terms of time and space, and did not accumulate contrast media with disease progression. This prevents from using routine T1 and T2 mode MRI for monitoring the course of the disease, its prognosis and treatment efficiency. We can recommend cerebral MRI for early diagnostics of ALS and also to rule out other diseases which may resemble ALS. However diagnostic value of 1.5 Tesla MRI is quite limited for diagnostics of classic ALS and its subtypes (PLS and PBP).

Full Text

Restricted Access

About the authors

Tima R Stuchevskaya

St Peterburg First Pavkov State Medical University


Dmitriy I Rudenko

St Peterburg First Pavkov State Medical University

MD, PhD, Dr. Med. Sc., prof. Dept. Neurology

Valery M Kazakov

St Peterburg First Pavkov State Medical University

MD, PhD, Dr. Med. Sc., prof., Dept. Neurology

Olga F Pozdnyakova

St Petersburg State Pediatric Medical University of Ministry of Health of the Russian Federation

MD, PhD physician of the Radiology Clinic

Alexandr V Pozdnyakov

St Petersburg State Pediatric Medical University of Ministry of Health of the Russian Federation

MD, PhD, Dr, Head of chair of medical Biophysics, head. Dept. of radiodiagnosis sbei HPE

Alexandr A Skoromets

St Peterburg First Pavkov State Medical University

academician of RAMS, MD, prof., head. Department of neurology and neurosurgery

Leonid A Tyutin

Russian scientific center of radiology and surgical technologies of the Ministry of health of the Russian Federation

MD, Professor of radiology, Deputy Director on scientific work


  1. Abrahams S, Leigh PH, Goldstein LH. Cognitive change in ALS: a prospective study. Neurology. 2005; 64(7):1222-6. doi: 10.1212/01.WNL. 0000156519.41681.27.
  2. Agosta F, Pagani E, Petrolini M, et al. Assessment of white matter tract damage in patients with amyotrophic lateral sclerosis: a diffusion tensor MR imaging tractography study. Am J Neuroradiol. 2010; 31:1457-61. doi: 10.3174/ajnr.A2105.
  3. Agosta F, Valsasina P, Absinta M, et al. Sensorimotor functional connectivity changes in amyotrophic lateral sclerosis. Cerebral Cortex. 2011;21:2291-8. doi: 10.1093/cercor/bhr002.
  4. Borasio G, Linke R, Schwarz J, et al. Dopaminergic deficit in amyotrophic lateral sclerosis assessed with [I-123] IPT single photon emission computed tomography. J Neurol Neurosurg Psychiat. 1998;65(2):263-5. doi: 10.1136/jnnp.65.2.263.
  5. Brooks B, Miller RG, Swash M, et al. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1:293-9. doi: 10.1080/146608200300079536.
  6. Cedarbaum JM, Stambler N, Malta E, et al. The ALSFRS-r: a revised ALS functional rating scale that incorporates assessments of respiratory function. J Neurol Sciences. 1999;169(1):13-21. doi: 10.1016/S0022-510X(99)00210-5.
  7. Comi G, Rovaris M, Leocani L. Review neuroimaging in amyotrophic lateral sclerosis. Eur J Neurol. 1999;6(6):629-37. doi: 10.1046/j.1468-1331.1999. 660629.x.
  8. Chan S, Shungu DC, Douglas-Akinwande A, et al. Motor Neuron Disease: Comparison of Single-Voxel Proton MR Spectroscopy of the Motor Cortex with MR Imaging of the Brain. Radiology. 1999;212(3):763-9. doi: 10.1148/radiology.212.3.r99au35763.
  9. Douaud G, Filippini N, Knight S, et al. Integration of structural and functional magnetic resonance imaging in amyotrophic lateral sclerosis. Brain. 2011;134 (12):3470-9. doi: 10.1093/brain/awr279.
  10. Filippini N, Donaud G, Mackay CE, et al. Corpus callosum involvement is a consistentfearture of amyotrophic lateral sclerosis. Neurology. 2010;75(18):1645-52. doi: 10.1212/WNL.0b013e3181fb84d1.
  11. Foerster BR, Welsh RC, Feldman EL. 25 years of neuroimaging in amyotrophic lateral sclerosis. Nature reviews. Neurology. 2013;9(9):513-24. doi: 10.1038/nrneurol.2013.153.
  12. Gerard G, Weisberg LA. MRI periventricular lesions in adults. Neurology. 1986;36(7):998-1001. doi: 10.1212/WNL.36.7.998.
  13. Geser F, Drandmeir NJ, Kwong LK, et al. Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis. Arch Neurol. 2008;65(5):636-41. doi: 10.1001/archneur.65.5.636.
  14. Goodin D, Rowley H, Olney R. Magnetic resonance imaging in amyotrophic lateral sclerosis. Ann Neurol. 1998;23(4):418-20. doi: 10.1002/ana.410230424.
  15. Gordon P, Delgadillo D, Piquard A, et al. The range and clinical impact of cognitive impairment in French patients with ALS: a cross-sectional study of neuropsychological test performance. Amyotroph Lateral Scler. 2011;12 (5):372-8. doi: 10.3109/17482968.2011.580847.
  16. Graham JM, Papadakis N, Evans J, et al. Diffusion tensor imaging for the assessment of upper motor neuron integrity in ALS. Neurology. 2004;63(11):2111-9. doi: 10.1212/01.WNL.0000145766.03057.E7.
  17. Guermazi A. Is high signal intensity in the cortispinal tract a sign of degeneration? Am J Neuroradiol. 1996; 17:801-2.
  18. Hecht M, Fellner F, Fellner C, et al. MRI-FLAIR images of the head show corticospinal tract alteration in ALS patients more frequently than T2-, T1-and proton-density-weighted images. J Neurol Sci. 2001;186(1-2):37-44. doi: 10.1016/S0022-510X(01)00503-2.
  19. Hong YH, Lee KW, Sung JJ, et al. Diffusion tensor MRI as a diagnostic tool of upper motor neuron involvement in amyotrophic lateral sclerosis. J Sci. 2004; 227(1):73-8. doi: 10.1016/j.jns.2004.08.014.
  20. Ince P, Evans J, Knopp M, et al. Corticospinal tract degeneration in the progressive muscular atrophy variant of ALS. Neurology. 2003;60:1252-8. doi: 10.1212/01.WNL.0000058901.75728.4E.
  21. Iwanaga K, Wakabayashi K, Honma Y, et al. Neuropathology of sporadic amyortophic lateral sclerosis of long duration. Clin Neuropathol. 1997;16(1):23-6.
  22. Jognson KA, Davis KR, Buonanno FS, et al. Comparison of magnetic resonance and roentgen ray computed tomography in dementia. Arch Neurol. 1987;44(10): 1075-80. doi: 10.1001/archneur.1987.00520220071020.
  23. Karlsborg M, Rosenbaum S, Wiegell MR, et al. Corticospinal tract degeneration and possible pathogenesis in ALS evaluated by MR diffusion tensor imaging. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004;5 (3):136-40. doi: 10.1080/14660820410018982.
  24. Kertesz A, Black SE, Tokar G, et al. Periventricular and subcortical hyperintensities on magnetic resonance imaging. Rims, caps and unidentified bright objects. Arch Neurol. 1988;45(4):404-8. doi: 10.1001/archneur.1988.00520280050015.
  25. Kew JJ, Leigh PN, Playford ED, et al. Cortical function in amyotrophic lateral sclerosis. A positron emission tomography study. Brain. 1993;116(3):655-80. doi: 10.1093/brain/116.3.655.
  26. Kolind S, Sharma R, Knight S, et al. Myelin imaging in amyotrophic and primary lateral sclerosis. Amyotroph. Lateral Scler Frontotemporal Degener. 2013;14(7-8): 562-73. doi: 10.3109/21678421.2013.794843.
  27. Lomen-Hoerth C, Murphy J, Langmore S, et al. Are amyotrophic lateral sclerosis patients cognitively normal? Neurology. 2003;60(7):1094-97. doi: 10.1212/01.WNL.0000055861.95202.8D.
  28. Lukes SA, Crooks LE, Aminoff MJ, et al. Nuclear magnetic resonance imaging in multiple sclerosis. Ann Neurol. 1983;13(6):592-601. doi: 10.1002/ana.410130603.
  29. Mitsumoto H, Ulug AM, Pullman SL, et al. Quantitative objective markers for upper and lower motor neuron dysfunction in ALS. Neurology. 2007;68(17):1402-10. doi: 10.1212/01.wnl.0000260065.57832.87.
  30. Peretti-Viton P, Azulay JP, Trefouret S, et al. MRI of the intracranial corticospinal tracts in amyotrophic and primary lateral sclerosis. Neuroradiol. 1999;41(10): 744-9. doi: 10.1007/s002340050836.
  31. Pradat P. New biological and radiological markers in amyotrophic lateral sclerosis. Press Med. 2009;38: 1843-51. doi: 10.1016/j.lpm.2009.01.022.
  32. Pradat PF, Bruneteau G. Classical and atypical clinical features in amyotrophic lateral sclerosis. Rev Neurol. 2006;162(2):4S17-4S24.
  33. Pyra T, Hui B, Hanstock C, et al. Combined structural and neurochemical evaluation of the corticospinal tract in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2010;11(1-2):157-65. doi: 10.3109/17482960902756473.
  34. Rippon G, Scarmeas N, Gordon PH, et al. An observational study of cognitive impairment in amyotrophic lateral sclerosis. Arch Neurol. 2006;63:345-52. doi: 10.1001/archneur.63.3.345.
  35. Sasaki S, Tsutsumi Y, Yamane K, et al. Sporadic amyotrophic lateral sclerosis with extensive neurological involvement. Acta Neuropathol. 1992;84(2):211-15. doi: 10.1007/BF00311398.
  36. Simon NG, Turner MR, Vucic S, et al. Quantifying Disease Progression in Amyotrophic Lateral Sclerosis. Ann Neurol. 2014;76(5)643-57. doi: 10.1002/ana.24273.
  37. Thorpe JW, Moseley IF, Hawkes CH, et al. Brain and spinal cord MRI in motor neuron disease. J Neurol Neurosurg Psychiat. 1996;61(3):314-7. doi: 10.1136/jnnp.61.3.314.
  38. Toosy AT, Werring DJ, Orell RW, et al. Diffusion tensor imaging detects corticospinal tract involvement at multiplelevels in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiat. 2003;74:1250-7. doi: 10.1136/jnnp.74.9.1250.
  39. Traynor B, Godd M, Corr B, et al. Clinical features of amyotrophic lateral sclerosis according to the El Escorial and Airlie House diagnostic criteria: a population-based study. Arch Neurol. 2000;57(8):1171-76. doi: 10.1001/archneur.57.8.1171.
  40. Turner MR, Cagnin A, Turkheimer FE, et al. Evidence of widespread cerebral microglial activation in amyotrophic lateral sclerosis: [11C] (R)-PK11195 positron emission tomography study. Neurobiol Dis. 2004;15: 601-9. doi: 10.1016/j.nbd.2003.12.012.
  41. Turner MR, Kiernan MC, Leigh P.N, et al. Biomarkers in amyotrophic lateral sclerosis. Lancet Neurol. 2009;8 (1):94-109. doi: 10.1016/S1474-4422(08)70293-X.
  42. Van den Berg-Vos RM, Visser J, Fanssen H, et al. Sporadic lower motor neuron disease with adult onset: classification of subtypes. Brain. 2003;126 (5):1036-47. doi: 10.1093/brain/awg117.
  43. Van Bogaert L. Syndrome de la calotte protin berantielle avec myoclonus localisees et troubles du sommeil. Rev Neurologique. 1926;45:977-88.
  44. Wechsler I, Davison C. Amyotrophic lateral sclerosis with mental symptoms. Arch Neurol Psychiat. 1932; 27:859-80. doi: 10.1001/archneurpsyc.1932.02230160100010.
  45. Zoccolella S, Beghi E, Palagano G, et al. Predictors of delay in the diagnosis and clinical trial entry of amyotrophic lateral sclerosis patients: a population-based study. J Neurol Sci. 2006;250:45-9. doi: 10.1016/j.jns.2006.06.027.



Abstract - 2163

PDF (Russian) - 639


Article Metrics

Metrics Loading ...



Copyright (c) 2016 Stuchevskaya T.R., Rudenko D.I., Kazakov V.M., Pozdnyakova O.F., Pozdnyakov A.V., Skoromets A.A., Tyutin L.A.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies