Skip to main content
AAN.com

Abstract

Background: A number of neurophysiologic and neuroimaging techniques have been evaluated in the research setting to assess upper motor neuron (UMN) damage in ALS. Changes in tissue structure in the CNS modify the diffusional behavior of water molecules, which can be detected by diffusion tensor MRI.
Objectives: To explore the hypothesis that degeneration of the motor fibers in ALS would be reflected by changes in the diffusion characteristics of the white matter fibers in the posterior limb of the internal capsule and that these changes could be detected by diffusion tensor MRI.
Methods: We studied 22 patients with El Escorial definite, probable, or possible ALS—11 with limb onset (mean age 54.5 ± 10.7 years) and 11 with bulbar onset (mean age 49.6 ± 11.7 years)—and compared them with 20 healthy, age-matched controls (mean age 46.0 ± 12.6 years). We assessed central motor conduction time (CMCT), threshold to stimulation, and silent period using transcranial magnetic stimulation. Diffusion tensor MRI was performed using a 1.5-T GE Signa system (Milwaukee, WI) fitted with Advanced NMR hardware and software capable of producing echo planar MR images. Data were acquired from seven coronal slices centered to include the posterior limb of the internal capsule. Maps of the mean diffusivity, fractional anisotropy, and T2-weighted signal intensity were generated.
Results: There were no differences between the subject groups on measures of CMCT, threshold to stimulation, and silent period. However, the CMCT correlated with clinical measures of UMN involvement. We found a significant increase in the mean diffusivity and reduction in fractional anisotropy along the corticospinal tracts between the three subject groups, most marked in the bulbar-onset group. The fractional anisotropy correlated with measures of disease severity and UMN involvement, whereas the mean diffusivity correlated with disease duration.
Conclusion: The results support the use of diffusion tensor MRI in detecting pathology of the corticospinal tracts in ALS.

Get full access to this article

View all available purchase options and get full access to this article.

References

1.
Claus D, Brunholzl C, Kerling FP, Henschel S. Transcranial magnetic stimulation as a diagnostic and prognostic test in amyotrophic lateral sclerosis. J Neurol Sci 1995;129:30–34.
2.
Barker AT, Freeston EL, Jalinous R, Jarrat JA. Clinical evaluation of conduction time measurement in central motor pathways using magnetic stimulation of the human brain. Lancet 1986;1:1325–1326.
3.
Berardelli A, Inghilleri M, Cruccu G, Mercuri B, Manfredi M. Electrical and magnetic transcranial stimulation in patients with corticospinal damage due to stroke or amyotrophic lateral sclerosis. Electroencephalogr Clin Neurophysiol 1991;81:389–396.
4.
Eisen A, Shybel W, Murphy K, Hoirch M. Cortical magnetic stimulation in amyotrophic lateral sclerosis. Muscle Nerve 1990;13:146–151.
5.
Hugon J, Lubeau M, Tabatard F, Chazot F, Vallat JM, Dumas M. Central motor conduction in amyotrophic lateral sclerosis. Ann Neurol 1987;22:544–546.
6.
Mills KR, Nithi KA. Peripheral and central motor conduction in amyotrophic lateral sclerosis. J Neurol Sci 1998;159:82–87.
7.
Mills KR, Nithi KA. Corticomotor threshold is reduced in early sporadic amyotrophic lateral sclerosis. Muscle Nerve 1997;20:1137–1141.
8.
Prout AJ, Eisen AA. The cortical silent period and amyotrophic lateral sclerosis. Muscle Nerve 1994;17:217–223.
9.
Pioro EP, Antel JP, Cashman NR, Arnold DL. Detection of cortical neuron loss in motor neuron disease by proton magnetic resonance spectroscopic imaging in vivo. Neurology 1994;44:1933–1938.
10.
Jones AP, Gunawardena WJ, Coutinho CMA, Gatt JA, Shaw IC, Mitchell JD. Preliminary results of proton magnetic resonance spectroscopy in motor neurone disease (amyotrophic lateral sclerosis). J Neurol Sci 1995;129:85–89.
11.
Gredal O, Rosenbaum S, Topp S, Karlsborg M, Strange P, Werdelin L. Quantification of brain metabolites in amyotrophic lateral sclerosis by localized proton magnetic resonance spectroscopy. Neurology 1997;48:878–881.
12.
Rooney WD, Miller RG, Gelinas D, Schuff N, Maudsley AA, Weiner MW. Decreased N-acetylaspartate in motor cortex and corticospinal tract in ALS. Neurology 1998;50:1800–1805.
13.
Block W, Karitzky J, Traber F, et al. Proton magnetic resonance spectroscopy of the primary motor cortex in patients with motor neuron disease. Arch Neurol 1998;55:931–936.
14.
Cwik V, Hanstock CC, Allen PS, Martin WRW. Estimation of brainstem neuronal loss in amyotrophic lateral sclerosis with in vivo proton magnetic resonance spectroscopy. Neurology 1998;50:72–77.
15.
Ellis CM, Simmons A, Andrews C, Dawson JM, Williams SCR, Leigh PN. A proton magnetic resonance spectroscopy study in ALS: correlation with clinical findings. Neurology 1998;51:1104–1109.
16.
Moseley ME, Cohen J, Mintorovitch L, et al. Early detection of regional cerebral ischemia in cats: comparison of diffusion and T2-weighted MRI and spectroscopy. Magn Reson Med 1990;14:330–346.
17.
Warach S, Gaa B, Siewert P, Wielopolski P, Edelman RR. Acute human stroke studied by whole brain echo planar diffusion weighted MRI. Ann Neurol 1995;37:231–241.
18.
Tien RD, Felsberg GJ, Friedman H, Brown M, MacFall J. MR imaging of high-grade cerebral gliomas: value of diffusion-weighted echo planar pulse sequences. AJR Am J Roentgenal 1994;162:671–677.
19.
Kalrik SJ, Gilbert JJ, Wong C, Vandervoot MK, Noseworthy JH. NMR studies in experimental allergic encephalomyelitis: factors which contribute to T1 and T2 values. Magn Reson Med 1990;14:1–11.
20.
Kalrik SJ, Strejan J, Gilbert JJ, Noseworthy JH. NMR studies in experimental allergic encephalomyelitis (EAE): normalization of T1 and T2 with parenchymal cellular infiltration. Neurology 1986;36:1112–1114.
21.
Heide AC, Richards TL, Alvord EC Jr, Peterson J, Rose LM. Diffusion imaging of experimental allergic encephalomyelitis. Magn Reson Med 1993;29:478–484.
22.
Moseley ME, Cohen Y, Kucharczyk J, et al. Diffusion-weighted MR imaging of anisotropic water diffusion in cat central nervous system. Radiology 1990;176:439–445.
23.
Chenvert TL, Brunberg JA, Pipe JG. Anisotropic diffusion in human white matter: demonstration with MR techniques in vivo. Radiology 1990;177:401–405.
24.
Doran M, Hajnal JV, Van Bruggen N, King MD, Young IR, Bydder GM. Normal and abnormal white matter tracts shown by MR imaging using directional diffusion-weighted sequences. J Comput Assist Tomogr 1990;14:856–873.
25.
Basser PJ, Mattiello J, Le Bihan D. MR diffusion tensor spectroscopy and imaging. Biophys J 1994;66:259–267.
26.
Basser PJ, Pierpaoli C. Microstructural and physiological features of tissue elucidated by quantitative diffusion-tensor MRI. J Magn Reson B 1996;111:209–219.
27.
Pierpaoli C, Basser PJ. Towards a quantitative assessment of diffusion anisotropy. Magn Reson Med 1996;36:893–906.
28.
Hazlewood CF, Rorschach HE, Lin C. Diffusion of water in tissues and MRI. Magn Reson Med 1991;19:214–216.
29.
Horsfield MA, Larsson HBW, Jones DK, Gass A. Diffusion magnetic resonance imaging in multiple sclerosis. J Neurol Neurosurg Psychiatry 1998;63 (suppl):S80–S84.
30.
Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. J Neurol Sci 1994;124 (suppl):S96–S107.
31.
Hillel AD, Miller RM, Yorkston K, McDonald E, Norris FH, Konikao N. Amyotrophic lateral sclerosis severity scale. Neuroepidemiology 1989;8:142–150.
32.
Eisen A, Entezari-Taher M, Stewart H. Cortical projections to spinal motoneurons: changes with aging and amyotrophic lateral sclerosis. Neurology 1996;46:1396–1404.
33.
Priori A. Clinical applications of silent period measurements. Advances in Occupational Medicine and Rehabilitation. 1996;2:91–97.
34.
Simmons A, Moore E, Williams SCR. Automated quality control for functional MRI studies. Neuroimage 1997;5:S466. Abstract.
35.
Simmons A, Arridge SR, Barker GJ, Williams SCR. Simulation of MRI cluster plots and application to neurological segmentation. Magn Reson Imaging 1996;14:73–92.
36.
Simmons A, Arridge SR, Barker GJ, Cluckie AJ, Tofts PS. Improvements to the quality of MRI cluster analysis. Magn Reson Imaging 1994;12:1191–1204.
37.
Peled S, Gudbjartsson H, Westin CF, Kikinis R, Jolesz FA. Magnetic resonance imaging shows orientation and asymmetry of white matter fibre tracts. Brain Res 1998;780:27–33.
38.
Larrson HBW, Thomsen C, Frederiksen J, et al. In vivo magnetic resonance diffusion measurements in the brain of patients with multiple sclerosis. Magn Reson Imaging 1992;10:7–12.
39.
Horsfield MA, Lai M, Webb SL, et al. Apparent diffusion coefficients in benign and secondary progressive multiple sclerosis by nuclear magnetic resonance. Magn Reson Med 1996;36:393–400.
40.
Lutsep HL, Albers GW, De Crespigny A, et al. Clinical utility of diffusion-weighted magnetic resonance imaging in the assessment of ischemic stroke. Ann Neurol 1997;41:574–580.
41.
Hanyu H, Shindo H, Kakizaki D, Abe K, Iwamoto T, Takasaki M. Increased water diffusion on cerebral white matter in Alzheimer’s disease. Gerontology 1997;43:343–351.
42.
Tievsky AL, Ptak T, Wu O, et al. Evaluation of MS lesions with full tensor diffusion-weighted imaging and anisotropy mapping. Proceedings of the fifth annual meeting of the International Society for Magnetic Resonance in Medicine; Vancouver, British Columbia, Canada. 1997;1:666. Abstract.
43.
Jones DK, Lythgoe D, Horsfield MA, Simmons A, Williams SCR, Markus HS. Characterization of white matter damage in ischemic leukoaraiosis with diffusion tensor MRI. Stroke 1999;30:393–397.
44.
Segawa F, Kishibayashi J, Kamada K, Sunohara N, Kinoshita M. MRI of paraventricular white matter lesions in amyotrophic lateral sclerosis: analysis by diffusion-weighted images. No To Shinkei 1994;46:835–840.
45.
Wu RH, Bruening R, Berchtenbreiter C, Borrasio T, Hueck A, Reiser M. Evaluation of diffusion-weighted imaging in patients with amyotrophic lateral sclerosis. Proceedings of the sixth annual meeting of International Society for Magnetic Resonance in Medicine; Sydney, Australia. 1998:1249. Abstract.
46.
Gideon P, Thomsen C, Henriksen O. Increased self-diffusion of brain water in normal aging. J Magn Reson Imaging 1994;4:185–188.
47.
Welch KMA, Windham J, Knight RA, et al. A model to predict the histopathology of human stroke using diffusion and T2-weighted magnetic resonance imaging. Stroke 1995;26:1983–1989.
48.
Warach S, Chien D, Li W, Ronthal M, Edelman RR. Fast magnetic resonance of diffusion-weighted imaging of acute stroke. Neurology 1992;42:1717–1723.

Information & Authors

Information

Published In

Neurology®
Volume 53Number 5September 1, 1999
Pages: 1051
PubMed: 10496265

Publication History

Received: November 30, 1998
Accepted: April 10, 1999
Published online: September 1, 1999
Published in print: September 1, 1999

Permissions

Request permissions for this article.

Authors

Affiliations & Disclosures

C.M. Ellis, MRCP
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.
A. Simmons, PhD
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.
D.K. Jones, MSc
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.
J. Bland, FRCP
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.
J.M. Dawson, FRCP, FRCR
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.
M.A. Horsfield, PhD
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.
S.C. R. Williams, PhD
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.
P.N. Leigh, PhD, FRCP
From the Department of Clinical Neurosciences (Drs. EllisSimmons, Bland, Dawson, Williams, and Leigh), Institute of Psychiatry, and Guy’s, King’s and St. Thomas’ School of Medicine; Neuroimaging Department (Drs. Simmons, Dawson, and Williams), Maudsley Hospital; and Division of Medical Physics (D.K. Jones and Dr. Horsfield), University of Leicester, Leicester Royal Infirmary, London, UK.

Notes

Address correspondence and reprint requests to Dr. Cathy M. Ellis, Department of Clinical Neurosciences, Institute of Psychiatry, De Crespigny Park, London SE5 8AF, UK.

Metrics & Citations

Metrics

Citation information is sourced from Crossref Cited-by service.

Citations

Download Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Select your manager software from the list below and click Download.

Cited By
  1. Myelin measurement in amyotrophic lateral sclerosis with synthetic MRI: A potential diagnostic and predictive method, Journal of the Neurological Sciences, 468, (123337), (2025).https://doi.org/10.1016/j.jns.2024.123337
    Crossref
  2. Features Suggestive of Coexisting Amyotrophic Lateral Sclerosis in Patients With Spinal Stenosis and Influence of Spinal Decompression, Cureus, (2024).https://doi.org/10.7759/cureus.51587
    Crossref
  3. Graph theory network analysis reveals widespread white matter damage in brains of patients with classic ALS, Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, (1-8), (2024).https://doi.org/10.1080/21678421.2024.2410281
    Crossref
  4. Coupling motor evoked potentials and brain [18F]FDG-PET in Amyotrophic Lateral Sclerosis: preliminary findings on disease severity, Neurobiology of Disease, 199, (106579), (2024).https://doi.org/10.1016/j.nbd.2024.106579
    Crossref
  5. Novel approaches to assessing upper motor neuron dysfunction in motor neuron disease/amyotrophic lateral sclerosis: IFCN handbook chapter, Clinical Neurophysiology, 163, (68-89), (2024).https://doi.org/10.1016/j.clinph.2024.04.010
    Crossref
  6. Potential of neuroimaging as a biomarker in amyotrophic lateral sclerosis: from structure to metabolism, Journal of Neurology, 271, 5, (2238-2257), (2024).https://doi.org/10.1007/s00415-024-12201-x
    Crossref
  7. Reconstructing the somatotopic organization of the corticospinal tract remains a challenge for modern tractography methods, Human Brain Mapping, 44, 17, (6055-6073), (2023).https://doi.org/10.1002/hbm.26497
    Crossref
  8. Pain-Related Coping Behavior in ALS: The Interplay between Maladaptive Coping, the Patient’s Affective State and Pain, Journal of Clinical Medicine, 11, 4, (944), (2022).https://doi.org/10.3390/jcm11040944
    Crossref
  9. Structure of the Motor Descending Pathways Correlates with the Temporal Kinematics of Hand Movements, Biology, 11, 10, (1482), (2022).https://doi.org/10.3390/biology11101482
    Crossref
  10. Biomarkers in the diagnosis of neurodegenerative diseases, RUDN Journal of Medicine, 26, 4, (431-440), (2022).https://doi.org/10.22363/2313-0245-2022-26-4-431-440
    Crossref
  11. See more
Loading...

View Options

Login options

Check if you have access through your login credentials or your institution to get full access on this article.

Personal login Institutional Login
Purchase Options

The neurology.org payment platform is currently offline. Our technical team is working as quickly as possible to restore service.

If you need immediate support or to place an order, please call or email customer service:

  • 1-800-638-3030 for U.S. customers - 8:30 - 7 pm ET (M-F)
  • 1-301-223-2300 for customers outside the U.S. - 8:30 - 7 pm ET (M-F)
  • [email protected]

We appreciate your patience during this time and apologize for any inconvenience.

View options

PDF and All Supplements

Download PDF and Supplementary Material

Full Text

View Full Text

Full Text HTML

View Full Text HTML

Media

Figures

Other

Tables

Share

Share

Share article link

Share