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January 31, 2023; 100 (5) Invited Article

Where's the Vision? The Importance of Visual Outcomes in Neurologic Disorders

The 2021 H. Houston Merritt Lecture

Sachi A. Patil, Scott Grossman, Rachel Kenney, Laura J. Balcer, Steven Galetta
First published December 15, 2022, DOI: https://doi.org/10.1212/WNL.0000000000201490
Sachi A. Patil
From the Department of Ophthalmology (S.A.P., L.J.B, S.G.), New York University Grossman School of Medicine, NY; Department of Neurology (S.G., L.J.B., S. Galetta), New York University Grossman School of Medicine, NY; Department of Radiology and Radiological Sciences (R.K.), Vanderbilt University School of Medicine, Nashville, TN; Department of Population Health (L.J.B.), New York University Grossman School of Medicine, NY.
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Scott Grossman
From the Department of Ophthalmology (S.A.P., L.J.B, S.G.), New York University Grossman School of Medicine, NY; Department of Neurology (S.G., L.J.B., S. Galetta), New York University Grossman School of Medicine, NY; Department of Radiology and Radiological Sciences (R.K.), Vanderbilt University School of Medicine, Nashville, TN; Department of Population Health (L.J.B.), New York University Grossman School of Medicine, NY.
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Rachel Kenney
From the Department of Ophthalmology (S.A.P., L.J.B, S.G.), New York University Grossman School of Medicine, NY; Department of Neurology (S.G., L.J.B., S. Galetta), New York University Grossman School of Medicine, NY; Department of Radiology and Radiological Sciences (R.K.), Vanderbilt University School of Medicine, Nashville, TN; Department of Population Health (L.J.B.), New York University Grossman School of Medicine, NY.
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Laura J. Balcer
From the Department of Ophthalmology (S.A.P., L.J.B, S.G.), New York University Grossman School of Medicine, NY; Department of Neurology (S.G., L.J.B., S. Galetta), New York University Grossman School of Medicine, NY; Department of Radiology and Radiological Sciences (R.K.), Vanderbilt University School of Medicine, Nashville, TN; Department of Population Health (L.J.B.), New York University Grossman School of Medicine, NY.
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Steven Galetta
From the Department of Ophthalmology (S.A.P., L.J.B, S.G.), New York University Grossman School of Medicine, NY; Department of Neurology (S.G., L.J.B., S. Galetta), New York University Grossman School of Medicine, NY; Department of Radiology and Radiological Sciences (R.K.), Vanderbilt University School of Medicine, Nashville, TN; Department of Population Health (L.J.B.), New York University Grossman School of Medicine, NY.
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Citation
Where's the Vision? The Importance of Visual Outcomes in Neurologic Disorders
The 2021 H. Houston Merritt Lecture
Sachi A. Patil, Scott Grossman, Rachel Kenney, Laura J. Balcer, Steven Galetta
Neurology Jan 2023, 100 (5) 244-253; DOI: 10.1212/WNL.0000000000201490

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Abstract

Neurologists have long recognized the importance of the visual system in the diagnosis and monitoring of neurologic disorders. This is particularly true because approximately 50% of the brain's pathways subserve afferent and efferent aspects of vision. During the past 30 years, researchers and clinicians have further refined this concept to include investigation of the visual system for patients with specific neurologic diagnoses, including multiple sclerosis (MS), concussion, Parkinson disease (PD), and conditions along the spectrum of Alzheimer disease (AD, mild cognitive impairment, and subjective cognitive decline). This review highlights the visual “toolbox” that has been developed over the past 3 decades and beyond to capture both structural and functional aspects of vision in neurologic disease. Although the efforts to accelerate the emphasis on structure-function relationships in neurologic disorders began with MS during the early 2000s, such investigations have broadened to recognize the need for outcomes of visual pathway structure, function, and quality of life for clinical trials of therapies across the spectrum of neurologic disorders. This review begins with a patient case study highlighting the importance using the most modern technologies for visual pathway assessment, including optical coherence tomography. We emphasize that both structural and functional tools for vision testing can be used in parallel to detect what might otherwise be subclinical events or markers of visual and, perhaps, more global neurologic decline. Such measures will be critical because clinical trials and therapies become more available across the neurologic disease spectrum.

Glossary

AD=
Alzheimer disease;
EDSS=
Expanded Disability Status Scale;
ETDRS=
Early Treatment Diabetic Retinopathy Study;
FA=
Friedrich ataxia;
GCIPL=
ganglion cell layer/inner plexiform layer;
GEE=
generalized estimating equation;
ICCs=
intraclass correlation coefficients;
ISI=
intersaccadic interval;
KD=
King-Devick;
MCI=
mild cognitive impairment;
MICK=
Mobile Integrated Cognitive Kit;
MS=
multiple sclerosis;
MSFC=
MS Functional Composite;
ON=
optic neuritis;
mTBI=
mild traumatic brain injury;
MULES=
Mobile Universal Lexicon Evaluation System;
NEI-VFQ-25=
25-Item National Eye Institute Visual Functioning Questionnaire;
OCT=
optical coherence tomography;
ONTT=
Optic Neuritis Treatment Trial;
PASAT=
Paced Serial Auditory Addition Task;
PD=
Parkinson disease;
RAN=
rapid automatized naming;
RNFL=
retinal nerve fiber layer;
SCD=
subjective cognitive decline;
SUN=
Staggered Uneven Number;
VOG=
video-oculography

Footnotes

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

  • Solicited and externally peer reviewed. The handling editor was Editor-in-Chief José Merino, MD, MPhil, FAAN.

  • Received April 26, 2022.
  • Accepted in final form September 14, 2022.
  • © 2022 American Academy of Neurology
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