Abstract Details

Title The Visual System in Huntington’s Disease

Topic Movement Disorders

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 10-049

Objective To evaluate a standardized protocol to assess eye movement and visual function in HD.

Background Saccadic eye movement abnormalities are among the earliest manifestations of Huntington’s disease (HD) but are difficult to quantify at the bedside, and afferent visual pathway involvement in HD is poorly characterized.

Design/Methods Participants with manifest HD (n=15) and healthy controls (n=13) performed the King-Devick test, a timed test of rapid number reading. Binocular high and low-contrast (2.5% and 1.25%) acuity was measured using Sloan Low-Contrast Letter charts, and pupillometric recordings were made using a handheld NeurOptics PLR-3000 pupillometer. The NEI VFQ-25 questionnaire with 10-item neuro-ophthalmic supplement was also completed. United Huntington’s Disease Rating Scale (UHDRS) motor score and other clinical and demographic variables were collected. Comparisons between manifest HD and controls were performed using linear regression adjusted for age and sex.

Results Mean King-Devick reading time was 106.3 seconds in manifest HD and 51.3 seconds in controls (p=0.00). Among manifest HD subjects, King-Devick time tended to be higher at higher UHDRS oculomotor, non-oculomotor, and total motor scores and at higher CAG repeat lengths, but these were not statistically significant. In unadjusted analyses, binocular high contrast acuity was 7 letters (one Snellen line equivalent) lower in manifest HD than controls (p=0.043), and this effect was similar at low-contrast acuity, but these associations were not statistically significant after adjusting for age. There were no differences in pupillary size, latency, velocity, or dilatation between manifest HD and controls. Individuals with manifest HD reported poorer peripheral vision than controls (p=0.011), but there were no differences in self-reported general health or vision.

Conclusions HD is associated with abnormal performance on the King-Devick test of rapid number naming and poorer self-reported peripheral vision. Analysis of contrast acuity and other measures were limited by sample size and will be further assessed in this ongoing study.

Authors/Disclosures
Ali G. Hamedani, MD, MHS (Hospital of the University of Pennsylvania) PRESENTER	Dr. Hamedani has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ArgenX. Dr. Hamedani has received personal compensation in the range of $0-$499 for serving as a Consultant for LoQus23. The institution of Dr. Hamedani has received research support from NIH. The institution of Dr. Hamedani has received research support from Biogen. The institution of Dr. Hamedani has received research support from Biohaven.
Tanya Bardakjian	Tanya Bardakjian has received personal compensation for serving as an employee of sarepta Therapeutics. Tanya Bardakjian has stock in Sarepta.
Pedro Gonzalez-Alegre, MD (Spark Therapeutics)	Dr. Gonzalez-Alegre has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Spark Therapeutics. Dr. Gonzalez-Alegre has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai Therapeutics. Dr. Gonzalez-Alegre has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuExcell. The institution of Dr. Gonzalez-Alegre has received research support from NIH/NINDS. Dr. Gonzalez-Alegre has received intellectual property interests from a discovery or technology relating to health care.

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