Saccadic Eye Movement Characteristics in Adult Niemann-Pick Type C Disease: Relationships with Disease Severity and Brain Structural Measures
AuthorAbel, LA; Bowman, EA; Velakoulis, D; Fahey, MC; Desmond, P; Macfarlane, MD; Looi, JCL; Adamson, CL; Walterfang, M
Source TitlePLOS ONE
PublisherPUBLIC LIBRARY SCIENCE
University of Melbourne Author/sWalterfang, Mark; Abel, Larry; Desmond, Patricia; Velakoulis, Dennis; BOWMAN, ELIZABETH; Bowman, Elizabeth
AffiliationOptometry And Vision Sciences
Document TypeJournal Article
CitationsAbel, L. A., Bowman, E. A., Velakoulis, D., Fahey, M. C., Desmond, P., Macfarlane, M. D., Looi, J. C. L., Adamson, C. L. & Walterfang, M. (2012). Saccadic Eye Movement Characteristics in Adult Niemann-Pick Type C Disease: Relationships with Disease Severity and Brain Structural Measures. PLOS ONE, 7 (11), https://doi.org/10.1371/journal.pone.0050947.
Access StatusAccess this item via the Open Access location
Open Access at PMChttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC3511378
C1 - Journal Articles Refereed
Niemann-Pick Type C disease (NPC) is a rare genetic disorder of lipid metabolism. A parameter related to horizontal saccadic peak velocity was one of the primary outcome measures in the clinical trial assessing miglustat as a treatment for NPC. Neuropathology is widespread in NPC, however, and could be expected to affect other saccadic parameters. We compared horizontal saccadic velocity, latency, gain, antisaccade error percentage and self-paced saccade generation in 9 adult NPC patients to data from 10 age-matched controls. These saccadic measures were correlated with appropriate MRI-derived brain structural measures (e.g., dorsolateral prefrontal cortex, frontal eye fields, supplemental eye fields, parietal eye fields, pons, midbrain and cerebellar vermis) and with measures of disease severity and duration. The best discriminators between groups were reflexive saccade gain and the two volitional saccade measures. Gain was also the strongest correlate with disease severity and duration. Most of the saccadic measures showed strongly significant correlations with neurophysiologically appropriate brain regions. While our patient sample is small, the apparent specificity of these relationships suggests that as new diagnostic methods and treatments become available for NPC, a broader range of saccadic measures may be useful tools for the assessment of disease progression and treatment efficacy.
KeywordsCentral Nervous System; Neurology and Neuromuscular Diseases; Human Pharmaceutical Treatments (e.g. Antibiotics); Nervous System and Disorders
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