Abstract Details

Title Virtual Reality Testing to Assess Cerebral Visual Impairment in X-linked Adrenoleukodystrophy

Topic Child Neurology and Developmental Neurology

Presentation(s) P1 - Poster Session 1 (8:00 AM-9:00 AM)

Poster/Presentation
Number 6-001

Objective

To characterize the extent of cerebral visual impairment (CVI) and visual processing deficits in X-linked adrenoleukodystrophy (X-ALD) through prospective virtual reality (VR) based testing.

Background X-ALD is an inherited single gene disorder that causes progressive inflammatory demyelination in 30-40% of boys and 20% of men. The demyelination predominantly affects the visual pathways in brain but symptoms of visual processing remain poorly characterized. To define the extent of CVI and visual processing deficits, we performed prospective testing using VR based visual search tasks in X-ALD patients with and without brain lesions.

Design/Methods VR environments combined with a Tobii 4C Eye Tracking System (90 Hz sampling rate) were used to assess visual search performance among patients with X-ALD in the Massachusetts General Brigham Leukodystrophy Clinic from April 2019-October 2021. All patients had confirmed X-ALD by genetic or biochemical testing. Two VR based tasks (one static and another dynamic) required searching for targets on a screen among varying levels of visual complexity.

Results

Virtual reality testing was completed in 28 X-ALD patients (CALD: n=20, non-CALD: n=8) and 38 controls. We found a reduced task performance determined by success rate, reaction time, and load sensitivity in ALD patients compared to controls even in patients who did not have a lesion. For the static task, the overall reaction time was 7% longer for the non-CALD and 15% longer for patients with CALD compared to controls. For the dynamic task, the overall reaction time was 25% longer for the non-CALD and 44% longer for the CALD group compared controls. The dynamic task revealed differences among X-ALD groups more strongly. Visual acuity did not correlate with task performance.

Conclusions

Higher order visual processing is impaired in patients with X-ALD. VR tasks have the potential to detect and measure deficits at the bedside, even when visual acuity is intact.

Authors/Disclosures
Melissa Bambery, Other (Massachusetts General Hospital) PRESENTER	Ms. Bambery has nothing to disclose.
Camille S. Corre, MD (University of Rochester Medical Center)	Dr. Corre has nothing to disclose.
Daniel C. Kelly	Mr. Kelly has nothing to disclose.
Amanda M. Nagy, MD (Massachusetts General Hospital)	Dr. Nagy has nothing to disclose.
	No disclosure on file
Claire Manley (Mass. Eye and Ear)	No disclosure on file
	No disclosure on file
	No disclosure on file
Florian Eichler, MD (Massachusetts General Hospital)	An immediate family member of Dr. Eichler has received personal compensation for serving as an employee of UpToDate. Dr. Eichler has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for SwanBio Therapeutics. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alnylam. Dr. Eichler has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Leal Therapeutics. Dr. Eichler has received personal compensation in the range of $100,000-$499,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Swan Bio. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. Dr. Eichler has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for UpToDate. Dr. Eichler has stock in SwanBio Therapeutics. The institution of Dr. Eichler has received research support from Minoryx Therpeutics. The institution of Dr. Eichler has received research support from ASPA Therapeutics. The institution of Dr. Eichler has received research support from bluebird bio. The institution of Dr. Eichler has received research support from Ionis Pharmaceuticals. Dr. Eichler has received intellectual property interests from a discovery or technology relating to health care.

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