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The clinical approach to the identification of higher-order visual dysfunction in neurodegenerative disease

  • Neuro-Ophthalmology (H. Moss, Section Editor)
  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

This review is intended to assist the reader in gaining the knowledge and skills necessary for the recognition and assessment of higher-order visual dysfunction due to neurodegenerative diseases including Alzheimer’s disease, dementia with Lewy bodies, Parkinson’s dementia, corticobasal degeneration, Creutzfeldt–Jakob disease, and the posterior cortical atrophy syndrome. Clinical problem-solving and pattern recognition must be developed and practiced to accurately diagnosis disturbances of higher-order visual function, and knowledge of higher-order visual brain regions and their visual syndromes forms the foundation for deciphering symptoms presented by patients and/or their care partners. Tests of higher-order visual dysfunction must be assembled by the clinician and assessment can take time and effort. The use of screening tests, follow-up visits, and formal neuropsychological referrals are critical components for accurate diagnosis and these principles are reviewed here.

Recent Findings

A recent survey of neuro-ophthalmologists revealed that over half of the respondents report that 5–10% of their new patient referrals carry a diagnosis of neurodegenerative disease and many patients were referred for visual symptoms of unknown cause. Despite over a century of discovery related to higher-order visual functions of the human brain, translation of discovery to the clinical assessment of patients has been slow or absent.

Summary

As with the approach to translational medicine in general, to see meaningful progress, an interdisciplinary approach is indispensable. The first step involves the application of discoveries from the field visual neuroscience by clinicians from the fields of ophthalmology, neurology, and neuropsychology, and from the disciplines of neuro-ophthalmology and behavioral neurology. The unmet need for recognition, assessment, and management of higher-order visual dysfunction in neurodegeneration is evident and clinicians can contribute to closing the gap by using the approach and the tools outlined in the review.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Barton JJS, Albonico A, Susilo T, Duchaine B, Corrow SL. Object recognition in acquired and developmental prosopagnosia. Cogn Neuropsychol. 2019;36(1–2):54–84.

    Article  Google Scholar 

  2. McCloskey M, Chaisilprungraung T. The value of cognitive neuropsychology: The case of vision research. Cogn Neuropsychol. 2017;34(7–8):412–9.

    Article  Google Scholar 

  3. de Best PB, Abulafia R, McKyton A, Levin N. Convergence along the visual hierarchy is altered in posterior cortical atrophy. Invest Ophthalmol Vis Sci. 2020;61(11):8.

    Article  Google Scholar 

  4. Holmes G. Ferrier Lecture. The organisation of the visual cortex in man. Proc R Soc Ser B. 1945;132:348–61.

    Google Scholar 

  5. Zeki S, Shipp S. The functional logic of cortical connections. Nature. 1988;335(6188):311–7.

    Article  CAS  Google Scholar 

  6. Ungerleider LG, Haxby J. “What” and “where” in the human brain. Curr Opin Neurobiol. 1994;4:157–65. Review of the relevant concepts and data supporting the two parallel pathways for higher order visual processing by one of the authors (Ungerleider) whose work led to discovery and support of the concept of what and where pathways.

    Article  CAS  Google Scholar 

  7. Goodale MA, Milner AD. Two visual pathways - Where have they taken us and where will they lead in future? Cortex. 2018;98:283–92.

    Article  Google Scholar 

  8. Pelak VS, Smyth SF, Boyer PJ, Filley CM. Computerized visual field defects in posterior cortical atrophy. Neurology. 2011;77(24):2119–22.

    Article  Google Scholar 

  9. Hawkins KM, Goyal AI, Sergio LE. Diffusion tensor imaging correlates of cognitive-motor decline in normal aging and increased Alzheimer’s disease risk. J Alzheimers Dis. 2015;44(3):867–78.

    Article  Google Scholar 

  10. Milner AD, Goodale MA. Visual pathways to perception and action. Prog Brain Res. 1993;95:317–37.

    Article  CAS  Google Scholar 

  11. Mapstone M, Steffenella TM, Duffy CJ. A visuospatial variant of mild cognitive impairment: getting lost between aging and AD. Neurology. 2003;60(5):802–8. Mapstone and colleagues provide evidence for higher order visual dysfunction as a presenting feature of early cognitive impairment for the Alzheimer’s disease clinical phenotype.

    Article  Google Scholar 

  12. Pelak VS, Hoyt WF. Symptoms of akinetopsia associated with traumatic brain injury and Alzheimer’s disease. Neuro-Ophthalmology. 2005;29:137–42.

    Article  Google Scholar 

  13. Yakubovich S, Israeli-Korn S, Halperin O, Yahalom G, Hassin-Baer S, Zaidel A. Visual self-motion cues are impaired yet overweighted during visual-vestibular integration in Parkinson’s disease. Brain Commun. 2020;2(1):035. Findings that support the notion that impaired perception of visual motion contributes to clinical manifestations of Parkinson’s disease.

    Article  Google Scholar 

  14. van der Ham IJM, Martens MAG, Claessen MHG, van den Berg E. Landmark Agnosia: Evaluating the Definition of Landmark-based Navigation Impairment. Arch Clin Neuropsychol. 2017;32(4):472–82.

    Article  Google Scholar 

  15. Crutch SJ, Schott JM, Rabinovici GD, et al. Consensus classification of posterior cortical atrophy. Alzheimers Dement. 2017;13(8):870–84. This manuscript provides current guidelines and criteria for diagnosing posterior cortical atrophy syndrome, which was based on consensus.

    Article  Google Scholar 

  16. Perry RJ, Hodges JR. Relationship between functional and neuropsychological performance in early Alzheimer disease. Alzheimer Dis Assoc Disord. 2000;14(1):1–10.

    Article  CAS  Google Scholar 

  17. Berliner JM, Kluger BM, Corcos DM, et al. Patient perceptions of visual, vestibular, and oculomotor deficits in people with Parkinson’s disease. Physiother Theory Pract. 2020;36(6):701–8.

    Article  Google Scholar 

  18. 2021 Alzheimer's disease facts and figures. Alzheimer's & dementia : the journal of the Alzheimer's Association. 2021;17(3):327-406.

  19. Sato T, Hanyu H, Hirao K, Shimizu S, Kanetaka H, Iwamoto T. Deep gray matter hyperperfusion with occipital hypoperfusion in dementia with Lewy bodies. Eur J Neurol. 2007;14(11):1299–301.

    Article  CAS  Google Scholar 

  20. Yokoi K, Nishio Y, Uchiyama M, Shimomura T, Iizuka O, Mori E. Hallucinators find meaning in noises: pareidolic illusions in dementia with Lewy bodies. Neuropsychologia. 2014;56:245–54.

    Article  Google Scholar 

  21. Liu Y, Pelak VS, van Stavern G, Moss HE. Higher Cortical Dysfunction Presenting as Visual Symptoms in Neurodegenerative Diseases. Front Neurol. 2020;11(679).

  22. Zeki S. A vision of the brain. Oxford; Boston: Blackwell Scientific Publications; 1993.

    Google Scholar 

  23. Ziegler GC, Haarmann A, Daniels C, Herr A. The Difficult Diagnosis of Posterior Cortical Atrophy in a 62-Year-Old Woman. J Geriatr Psychiatry Neurol. 2020;33(1):59–64.

    Article  Google Scholar 

  24. Olds JJ, Hills WL, Warner J, et al. Posterior Cortical Atrophy: Characteristics From a Clinical Data Registry. Front Neurol. 2020;11:358. This study provides clinical characteristics for the PCA syndrome in presentation to neuro-ophthalmology from a multicenter cohort.

    Article  Google Scholar 

  25. Alegret M, Boada-Rovira M, Vinyes-Junque G, et al. Detection of visuoperceptual deficits in preclinical and mild Alzheimer’s disease. J Clin Exp Neuropsychol. 2009;31(7):860–7.

    Article  Google Scholar 

  26. Giannakopoulos P, Gold G, Duc M, Michel JP, Hof PR, Bouras C. Neuroanatomic correlates of visual agnosia in Alzheimer’s disease: a clinicopathologic study. Neurology. 1999;52(1):71–7.

    Article  CAS  Google Scholar 

  27. McKhann GM, Knopman DS, Chertkow H, et al. The diagnosis of dementia due to Alzheimer’s disease: recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer’s disease. Alzheimer’s Dementia. 2011;7(3):263–9.

    Article  Google Scholar 

  28. McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology. 2017;89(1):88–100.

    Article  Google Scholar 

  29. Emre M, Aarsland D, Brown R, et al. Clinical diagnostic criteria for dementia associated with Parkinson’s disease. Mov Disord. 2007;22(12):1689–707 (quiz 1837).

    Article  Google Scholar 

  30. Coughlin DG, Hurtig HI, Irwin DJ. Pathological Influences on Clinical Heterogeneity in Lewy Body Diseases. Mov Disord. 2020;35(1):5–19.

    Article  Google Scholar 

  31. Armstrong MJ, Litvan I, Lang AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496–503.

    Article  Google Scholar 

  32. Zerr I, Kallenberg K, Summers DM, et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009;132(Pt 10):2659–68.

    Article  CAS  Google Scholar 

  33. Manix M, Kalakoti P, Henry M, et al. Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy. Neurosurg Focus. 2015;39(5):E2.

    Article  Google Scholar 

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Authors and Affiliations

  1. Departments of Neurology and Ophthalmology, Divisions of Neuro-Ophthalmology and Behavioral Neurology, University of Colorado School of Medicine, Aurora, CO, USA

    Victoria S. Pelak

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Conflicts of interests/Competing interests

Unrelated to this article, support has been received by the author in form of royalties to the author from UpToDate, Inc, honoraria for committee work and invited presentation (Alzheimer’s Association and Spectrum Health Neurosciences, respectively); payment to the author’s institution for clinical trials (Biogen and Eli Lilly and Company) and work as a co-investigator on an observational study (NIH R01 grant) and principal investigator (Visual Snow Initiative).

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Pelak, V.S. The clinical approach to the identification of higher-order visual dysfunction in neurodegenerative disease. Curr Neurol Neurosci Rep 22, 229–242 (2022). https://doi.org/10.1007/s11910-022-01186-7

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