Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Diversity, Equity, & Inclusion (DEI)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology Future Forecasting Series
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit New Manuscript
    • Submit Revised Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Diversity, Equity, & Inclusion (DEI)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology Future Forecasting Series
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit New Manuscript
    • Submit Revised Manuscript
    • Author Center
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Neurology Video Journal Club
  • Residents & Fellows

User menu

  • Subscribe
  • My Alerts
  • Log in

Search

  • Advanced search
Neurology
Home
The most widely read and highly cited peer-reviewed neurology journal
  • Subscribe
  • My Alerts
  • Log in
Site Logo
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Neurology Video Journal Club
  • Residents & Fellows

Share

November 08, 2016; 87 (19) Resident and Fellow Section

Pearls & Oy-sters: Visual agnosia

An overlooked cortical sign

Bruno Bergmans, Olivier Deryck, Rose Bruffaerts
First published November 7, 2016, DOI: https://doi.org/10.1212/WNL.0000000000003306
Bruno Bergmans
From the Neurology Department (B.B., O.D.), AZ Sint-Jan Brugge-Oostende AV, Brugge; Laboratory for Cognitive Neurology (R.B.), Department of Neurosciences, KU Leuven, University of Leuven; and Neurology Department (R.B.), University Hospitals Leuven, Belgium.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Olivier Deryck
From the Neurology Department (B.B., O.D.), AZ Sint-Jan Brugge-Oostende AV, Brugge; Laboratory for Cognitive Neurology (R.B.), Department of Neurosciences, KU Leuven, University of Leuven; and Neurology Department (R.B.), University Hospitals Leuven, Belgium.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Rose Bruffaerts
From the Neurology Department (B.B., O.D.), AZ Sint-Jan Brugge-Oostende AV, Brugge; Laboratory for Cognitive Neurology (R.B.), Department of Neurosciences, KU Leuven, University of Leuven; and Neurology Department (R.B.), University Hospitals Leuven, Belgium.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Full PDF
Citation
Pearls & Oy-sters: Visual agnosia
An overlooked cortical sign
Bruno Bergmans, Olivier Deryck, Rose Bruffaerts
Neurology Nov 2016, 87 (19) e237-e238; DOI: 10.1212/WNL.0000000000003306

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
1429

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

PEARL

  • Dorsal simultanagnosia is a sign of biparietal cortical pathology. When found in combination with a hypokinetic-rigid syndrome, corticobasal degeneration should be considered.

OY-STERS

  • Patients will often not complain of subtle visual processing deficits. If neuropsychological testing is not performed, these deficits will go unnoticed.

  • Confrontation naming can be impaired for different reasons: the presence of nonfluent aphasia in this patient cannot explain the partonomic errors.

We report a 77-year-old patient with visual agnosia as a cortical sign of neurodegenerative disease.

The patient presented with balance problems and frequent falls since age 72 and subsequent nonfluent aphasia. Clinical examination revealed a supranuclear vertical gaze palsy and a symmetrical hypokinetic-rigid syndrome. MRI of the cerebrum demonstrated enlargement of the left Sylvian fissure and bilateral intraparietal sulci as well as a hummingbird sign (figure 1, A–D). DaTscan showed bilateral reduced uptake (figure 1E). This clinical phenotype is suggestive of progressive supranuclear palsy.1

Figure 1
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 1 MRI and DaTscan

(A) Coronal MRI slice: asymmetric enlargement of the left Sylvian fissure (arrow). (B) Coronal MRI slices: enlargement of the bilateral intraparietal sulci (arrows). Note that the temporal cortex is relatively spared. (C) Axial MRI slice: enlargement of the bilateral intraparietal sulci (arrows). (D) Sagittal MRI slice: hummingbird sign. (E) DaTscan: bilateral reduced uptake.

Neuropsychological testing revealed executive dysfunction. The patient, whose vision was corrected to normal with glasses, scored 8/15 on an abbreviated version of the Boston Naming Test. He produced 2 partonomic errors: instead of a tree, he saw leaves, instead of a flower, he saw a branch. He called a hammock a bed, a camel a giraffe, a mask a clown. He could not identify a funnel and tongs. Naming errors due to nonfluent aphasia are typically phonetic errors. Partonomic errors during confrontation naming are visual in nature and indicate a deficit of recognition rather than of name retrieval. This is suggestive of visual agnosia.

When asked to copy the drawings of the Boston Naming Test, the patient omitted several salient surface features (figure 2). The inability of our patient to copy drawings suggests that he has dorsal simultanagnosia rather than ventral simultanagnosia. Both types of simultanagnosia lead to object identification deficits. In dorsal simultanagnosia, the naming errors occur because the patient does not perceive the picture in its entirety. Therefore, a patient with dorsal simultanagnosia will be impaired when asked to copy a drawing. In ventral simultanagnosia, the picture is perceived in its entirety, but it cannot be identified as a whole. This explains why a patient with ventral simultanagnosia can copy a drawing.2

Figure 2
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 2 Copy drawing of the Boston Naming Test

Several visuoperceptual errors were documented when the patient copied the Boston Naming Test. The left column shows the probe picture, the right column shows the drawing made by the patient. Flower: placement error: no leaves are found on the left side. House: placement error of the top right window; salient features, e.g., chimneys, were not copied; the shape of the roof is inaccurate. Tongs: faulty perspective, inaccurately overlapping shapes. Whistle: faulty perspective, surface features are omitted.

Dorsal simultanagnosia is part of a Bálint syndrome, together with optic ataxia and ocular apraxia. Dorsal simultanagnosia can arise due to biparietal damage secondary to structural pathology or neurodegenerative disease, e.g., corticobasal degeneration, posterior cortical atrophy, or Creutzfeldt-Jakob disease. In our patient, the working hypothesis is corticobasal degeneration.3 Given the initial clinical presentation suggestive of progressive supranuclear palsy, our patient adds to the growing phenotypic spectrum of tauopathies.4

AUTHOR CONTRIBUTIONS

B.B.: examination of the patient, revision of the manuscript for important intellectual content. O.D.: examination of the patient, revision of the manuscript for important intellectual content. R.B.: drafting of the manuscript, revision of the manuscript for important intellectual content.

STUDY FUNDING

No targeted funding reported.

DISCLOSURE

The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

ACKNOWLEDGMENT

The authors thank Patrick Santens for comments on this case and Jan Casselman and Frank De Geeter for providing the MRI and DaTscan images.

Footnotes

  • Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • © 2016 American Academy of Neurology

REFERENCES

  1. 1.↵
    1. Josephs KA,
    2. Duffy JR
    . Apraxia of speech and nonfluent aphasia: a new clinical marker for corticobasal degeneration and progressive supranuclear palsy. Curr Opin Neurol 2008;21:688–692.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Farah MJ
    . Visual Agnosia, 2nd ed. Cambridge, MA: MIT Press; 2004.
  3. 3.↵
    1. Armstrong M,
    2. Litvan I,
    3. Lang A, et al
    . Criteria for the diagnosis of corticobasal degeneration. Neurology 2013;80:496–503.
    OpenUrlAbstract/FREE Full Text
  4. 4.↵
    1. Williams DR,
    2. Lees AJ
    . Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol 2009;8:270–279.
    OpenUrlCrossRefPubMed
View Abstract

Letters: Rapid online correspondence

No comments have been published for this article.
Comment

REQUIREMENTS

If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org

Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.

If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.

Submission specifications:

  • Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
  • Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
  • Submit only on articles published within 6 months of issue date.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Publishing Agreement Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
    • PEARL
    • OY-STERS
    • AUTHOR CONTRIBUTIONS
    • STUDY FUNDING
    • DISCLOSURE
    • ACKNOWLEDGMENT
    • Footnotes
    • REFERENCES
  • Figures & Data
  • Info & Disclosures
Advertisement

SARS-CoV-2 Vaccination Safety in Guillain-Barré Syndrome, Chronic Inflammatory Demyelinating Polyneuropathy, and Multifocal Motor Neuropathy

Dr. Jeffrey Allen and Dr. Nicholas Purcell

► Watch

Related Articles

  • No related articles found.

Topics Discussed

  • Agnosia
  • Progressive supranuclear palsy
  • Corticobasal degeneration
  • Assessment of cognitive disorders/dementia

Alert Me

  • Alert me when eletters are published

Recommended articles

  • Articles
    CSF biomarkers in posterior cortical atrophy
    J. Seguin, M. Formaglio, A. Perret-Liaudet et al.
    Neurology, April 27, 2011
  • Articles
    The cognitive profile of posterior cortical atrophy
    Paul McMonagle, Fiona Deering, Yaniv Berliner et al.
    Neurology, February 13, 2006
  • Articles
    Progressive posterior cortical dysfunction
    A clinicopathologic series
    J. A. Renner, J. M. Burns, C. E. Hou et al.
    Neurology, October 11, 2004
  • Articles
    Neuroanatomic correlates of visual agnosia in Alzheimer’s disease
    A clinicopathologic study
    P. Giannakopoulos, G. Gold, M. Duc et al.
    Neurology, January 01, 1999
Neurology: 100 (13)

Articles

  • Ahead of Print
  • Current Issue
  • Past Issues
  • Popular Articles
  • Translations

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Activate a Subscription
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Education
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology | Print ISSN:0028-3878
Online ISSN:1526-632X

© 2023 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise