Skip to main content
Advertisement

Main menu

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • Advancing NeuroMRI with High-Relaxivity Contrast Agents
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home

User menu

  • Alerts
  • Log in

Search

  • Advanced search
American Journal of Neuroradiology
American Journal of Neuroradiology

American Journal of Neuroradiology

ASHNR American Society of Functional Neuroradiology ASHNR American Society of Pediatric Neuroradiology ASSR
  • Alerts
  • Log in

Advanced Search

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • Advancing NeuroMRI with High-Relaxivity Contrast Agents
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home
  • Follow AJNR on Twitter
  • Visit AJNR on Facebook
  • Follow AJNR on Instagram
  • Join AJNR on LinkedIn
  • RSS Feeds

AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates


Improved Turnaround Times | Median time to first decision: 12 days

Research ArticleBrain

Cerebral Cortical and White Matter Lesions in Amyotrophic Lateral Sclerosis with Dementia: Correlation with MR and Pathologic Examinations

E. Matsusue, S. Sugihara, S. Fujii, T. Kinoshita, T. Nakano, E. Ohama and T. Ogawa
American Journal of Neuroradiology September 2007, 28 (8) 1505-1510; DOI: https://doi.org/10.3174/ajnr.A0605
E. Matsusue
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
S. Sugihara
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
S. Fujii
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
T. Kinoshita
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
T. Nakano
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
E. Ohama
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
T. Ogawa
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • Responses
  • References
  • PDF
Loading

Article Figures & Data

Figures

  • Tables
  • Fig 1.
    • Download figure
    • Open in new tab
    • Download powerpoint
    Fig 1.

    Case 1: Coronal T2-weighted MR images obtained 1 year after the onset of ALSD (A–C). AtNOphy is seen in the anteromedial part of the bilateral temporal lobes, especially on the right side, but not apparent in the frontal lobes. Postmortem coronal T2-weighted MR images (D–F) show asymmetric atrophy of the temporal lobes, more severe on the right side. T2 hyperintensities are also seen in the anteromedial part of the right temporal white matter. G, The right temporal lobe in (D) shows linear hyperintensity in the subcortical white matter (arrowheads). H, A Klüver-Barrera-stained section corresponding to boxed area in (G) shows laminar pallor of the subcortical white matter (arrowheads). I, Boxed area in (H) shows spongiform changes in the cortex (arrows). J, Histologic examination of the area in (I) shows spongiform changes with neuronal loss and gliosis. H, Klüver-Barrera stain x 20. I, hematoxylin-eosin, x 100. J, GFAP, x 200. K, Right temporal lobe in (E) shows hyperintensities in the medial part of the temporal white matter, especially in the underlying U-fibers (arrowheads). L, A Klüver-Barrera-stained section corresponding to (K) reveals pallor in the medial part of the temporal white matter, especially in the underlying U-fibers (arrowheads). M, A Holzer-stained section corresponding to (K) reveals gliosis in the medial part of the temporal white matter, especially in the underlying U-fibers (arrowheads). N, O, Histologic examination of normal signal intensities on MR image (K–M, circles) shows no apparent degenerative changes. P–R, Histologic examination of the U-fibers (arrows) in (K–M) shows loss of myelin (P) and axons (Q) with moderate gliosis (R). N, P, Klüver-Barrera stain. O, Q, Bielschowsky stain. R, GFAP stain. N, O, P, Q, R, x 200.

  • Fig 2.
    • Download figure
    • Open in new tab
    • Download powerpoint
    Fig 2.

    Case 2: Axial (A–C) and coronal (D–F) T2-weighted MR images obtained 4 months before death. Asymmetric cerebral atrophy is seen in the frontal and temporal lobes, more marked on the right side. Atrophy is more prominent in the anteromedial part of the temporal lobes. Focal and faint hyperintensities are seen in the subcortical white matter in the anteromedial part of the right temporal lobe (arrowheads in E). No definite T2-hyperintensities are seen in the corticospinal tracts in the internal capsules, cerebral peduncles, and pontine base. Change in signal intensity of the substantia nigra is also not seen.

  • Fig 3.
    • Download figure
    • Open in new tab
    • Download powerpoint
    Fig 3.

    Case 3: Postmortem axial (A–C) and coronal (D–F) T2-weighted MR images. Cerebral atrophy is seen in the frontal and temporal lobes, especially in the anteromedial part of the temporal lobes. Confluent and diffuse hyperintensities are seen in the frontal and temporal white matter. No definite T2-hyperintensities are seen in the corticospinal tracts and substantia nigra. G, The left temporal lobe in (D) shows hyperintensity in the subcortical and deep white matter. H, A Klüver-Barrera-stained section corresponding to (G) reveals pallor in the temporal subcortical and deep white matter. Histologic examination of hyperintensity on MR image (G, circle) shows severe loss of myelin (I) and axons (J), which reveals tissue rarefaction. I, Klüver-Barrera stain. K, Bielschowsky stain. I, K, x 200.

Tables

  • Figures
    • View popup
    Table 1:

    Summary of clinical and pathologic findings in three cases of ALSD

    Case No.Age/SexDuration of Illness (yrs/mo)Clinical FindingsBrain Weight (grams)Histologic Findings
    169/M2/1
    • Mild dementia

    • Aphasia

    • Dysphagia

    • Muscular atrophy of tongue and shoulder muscle

    1,280
    • Mild degeneration of corticospinal tracts in brain stem and spinal cord.

    • Neuronal loss with gliosis in lower motor neurons.

    • Bunina bodies in remaining motor neurons in spinal cord.

    • Microvacuoles, mild neuronal loss, and gliosis in second and third layers of frontal and temporal cortices.

    • Spongiform changes and mild gliosis in frontal and temporal subcortical white matter.

    • Moderate loss of pigmented neurons with gliosis in substantia nigra.

    • Ubiquitin-positive cytoplasmic inclusions in hippocampal dentate granule cells and small neurons in parahippocampal cortex.

    250/M5/4
    • Dementia

    • Dysphagia

    • Fasciculation and atrophy of tongue

    • Atrophy of upper and lower extremities, particularly distal part of upper extremities

    1,400
    • Mild degeneration of corticospinal tracts in brain stem and spinal cord.

    • Neuronal loss with gliosis in lower motor neurons.

    • Bunina bodies in remaining motor neurons in spinal cord.

    • Microvacuoles, mild neuronal loss, and gliosis in second and third layers of frontal and temporal cortices.

    • Spongiform changes and mild gliosis in frontal and temporal subcortical white matter.

    • Moderate loss of pigmented neurons with gliosis in substantia nigra.

    • Ubiquitin-positive cytoplasmic inclusions in hippocampal dentate granule cells and small neurons in parahippocampal cortex.

    369/M5
    • Apathy

    • Depression

    • Change of character

    • Dementia

    • Atrophy of upper and lower extremities

    1,200
    • Mild degeneration of corticospinal tracts in brain stem and spinal cord.

    • Neuronal loss with gliosis in lower motor neurons.

    • Bunina bodies in remaining motor neurons in spinal cord.

    • Microvacuoles, mild neuronal loss, and gliosis in second and third layers of frontal and temporal cortices.

    • Tissue rarefaction in subcortical and deep white matters of frontal and temporal lobes.

    • Moderate loss of pigmented neurons with gliosis in substantia nigra.

    • No ubiquitin-positive cytoplasmic inclusions in hippocampal dentate granule cells and other cortical neurons.

    • Note:—ALSD indicates amyotrophic lateral sclerosis with dementia.

    • View popup
    Table 2:

    Summary of antemortem and postmortem MR imaging findings in three cases of ALSD

    Case No.Age/SexDuration of Illness (yrs/mo)Antemortem T2-Weighted Imaging FindingsPostmortem T2-Weighted Imaging Findings
    169/M2/1
    • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

    • Mild atrophy of frontal lobes.

    • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

    • No signal changes in cerebral white matter.

    • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra (obtained 1 year before death).

    • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

    • Mild atrophy of frontal lobes.

    • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

    • Hyperintensities in anteromedial white matter of temporal lobes. Linear subcortical hyperintensities in temporal lobes, more marked on right side.

    • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.

    250/M5/4
    • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

    • Mild atrophy of frontal lobes.

    • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

    • Faint hyperintensities in subcortical white matter in anteromedial part of right temporal lobe.

    • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra (obtained 4 months before death)

    .
    • Atrophy in anteromedial part of bilateral temporal lobes, more marked on right side.

    • Mild atrophy of frontal lobes.

    • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

    • Hyperintensities in subcortical white matter of frontal and temporal lobes.

    • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.

    369/M5Not available
    • Symmetric atrophy of frontal and temporal lobes, more marked in anteromedial part of temporal lobes.

    • Atrophy of cerebral cortices, but no signal changes in cerebral cortices.

    • Hyperintensities in subcortical and deep white matter of frontal and temporal lobes.

    • No atrophy or signal changes in corticospinal tracts in cerebrum and brain stem and in substantia nigra.

    • Note:—ALSD indicates amyotrophic lateral sclerosis with dementia.

PreviousNext
Back to top

In this issue

American Journal of Neuroradiology: 28 (8)
American Journal of Neuroradiology
Vol. 28, Issue 8
September 2007
  • Table of Contents
  • Index by author
Advertisement
Print
Download PDF
Email Article

Thank you for your interest in spreading the word on American Journal of Neuroradiology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Cerebral Cortical and White Matter Lesions in Amyotrophic Lateral Sclerosis with Dementia: Correlation with MR and Pathologic Examinations
(Your Name) has sent you a message from American Journal of Neuroradiology
(Your Name) thought you would like to see the American Journal of Neuroradiology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Cite this article
E. Matsusue, S. Sugihara, S. Fujii, T. Kinoshita, T. Nakano, E. Ohama, T. Ogawa
Cerebral Cortical and White Matter Lesions in Amyotrophic Lateral Sclerosis with Dementia: Correlation with MR and Pathologic Examinations
American Journal of Neuroradiology Sep 2007, 28 (8) 1505-1510; DOI: 10.3174/ajnr.A0605

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
0 Responses
Respond to this article
Share
Bookmark this article
Cerebral Cortical and White Matter Lesions in Amyotrophic Lateral Sclerosis with Dementia: Correlation with MR and Pathologic Examinations
E. Matsusue, S. Sugihara, S. Fujii, T. Kinoshita, T. Nakano, E. Ohama, T. Ogawa
American Journal of Neuroradiology Sep 2007, 28 (8) 1505-1510; DOI: 10.3174/ajnr.A0605
del.icio.us logo Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Purchase

Jump to section

  • Article
    • Abstract
    • Materials and Methods
    • Results
    • Discussion
    • Conclusion
    • References
  • Figures & Data
  • Info & Metrics
  • Responses
  • References
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • The Present and the Future of Neuroimaging in Amyotrophic Lateral Sclerosis
  • Crossref
  • Google Scholar

This article has not yet been cited by articles in journals that are participating in Crossref Cited-by Linking.

More in this TOC Section

  • Predictors of Reperfusion in Patients with Acute Ischemic Stroke
  • Enhanced Axonal Metabolism during Early Natalizumab Treatment in Relapsing-Remitting Multiple Sclerosis
  • Progression of Microstructural Damage in Spinocerebellar Ataxia Type 2: A Longitudinal DTI Study
Show more Brain

Similar Articles

Advertisement

Indexed Content

  • Current Issue
  • Accepted Manuscripts
  • Article Preview
  • Past Issues
  • Editorials
  • Editor's Choice
  • Fellows' Journal Club
  • Letters to the Editor
  • Video Articles

Cases

  • Case Collection
  • Archive - Case of the Week
  • Archive - Case of the Month
  • Archive - Classic Case

More from AJNR

  • Trainee Corner
  • Imaging Protocols
  • MRI Safety Corner
  • Book Reviews

Multimedia

  • AJNR Podcasts
  • AJNR Scantastics

Resources

  • Turnaround Time
  • Submit a Manuscript
  • Submit a Video Article
  • Submit an eLetter to the Editor/Response
  • Manuscript Submission Guidelines
  • Statistical Tips
  • Fast Publishing of Accepted Manuscripts
  • Graphical Abstract Preparation
  • Imaging Protocol Submission
  • Evidence-Based Medicine Level Guide
  • Publishing Checklists
  • Author Policies
  • Become a Reviewer/Academy of Reviewers
  • News and Updates

About Us

  • About AJNR
  • Editorial Board
  • Editorial Board Alumni
  • Alerts
  • Permissions
  • Not an AJNR Subscriber? Join Now
  • Advertise with Us
  • Librarian Resources
  • Feedback
  • Terms and Conditions
  • AJNR Editorial Board Alumni

American Society of Neuroradiology

  • Not an ASNR Member? Join Now

© 2025 by the American Society of Neuroradiology All rights, including for text and data mining, AI training, and similar technologies, are reserved.
Print ISSN: 0195-6108 Online ISSN: 1936-959X

Powered by HighWire